Journal of Pakistan Association of
Dermatologists
Volume 13, Number 4 October-December, 2003
Editor
Ijaz Hussain
Associate Editors
Farhana Muzaffar
Zahida Rani
Faria Asad
Editorial Board
Abdul Aziz Memon
Abdul Ghafoor Qamar
Ahsan Hameed
Akhtar Waheed Khan
Ali Khan Tareen
Anjum Kanjee
Anoop Kumar
Arfan ul Bari
Arif Maan
Ashfaq Ahmed Khan
Atif Hasnain Kazmi
Badr S. Dhanani
Farooq Soomro
Ghulam Mujtaba
Hasina Thawerani
Iqbal Akhtar Khan
Iqbal Chowdhry
Iqbal Tareen
Jameel A. Shaheen
Jawaid Anwar
Khadimullah Kakakhel
Khalid Hashmi
Khalid Hussain
Khalid Makhdoomi
Khawar Khurshid
Liaqat Ali Khan
Mansoor Dilnawaz
Muhammad Jahangir
Naeem Iqbal
Naseema Kapadia
Nasser Rashid Dar
Nizamul Hussain
Pervaiz Iqbal
Raza Jaffery
Raza Muhammad Khan
S. M. Azam Bokhari
S.M. Shamim
Sabrina Suhail Pal
Sajid Mushtaq
Satish S. Savant
Shahbaz A. Janjua
Sharaf Ali Shah
Shehab Afzal Beg
Shehbaz Aman
Simeen Ber Rahman
Tahir Anees
Tahir Jamil Ahmad
Tahir Saeed Haroon
Tariq Rashid
Tariq Zaman
Yasmeena Khan
Zarnaz Wahid
Zohra Zaidi
Zubair Memon
Publication Manager
Mr. Omar Abdul Aziz
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Copyright
Non-Langerhans cell histiocytoses
Farhana Muzaffar
 2003 Any material published in JPAD is copyright of Pakistan Association of
Dermatologists.
2
Journal of Pakistan Association of
Dermatologists
Volume 13, Number 4 October-December, 2003
Contents
Editorial
Non-Langerhans cell histiocytoses
Farhana Muzaffar
161
Original articles
Childhood eczema: a comparative analysis
Ijaz Ahmed, Mujeeb Ansari, Kashef Malick
164
Role of serodiagnosis in cutaneous leishmaniasis
Simeen ber Rahman, Arfan ul Bari, Haroon ur Rashid
171
Evaluation of sensitivity of Tzanck smear in pemphigus
Jameel A. Shaheen, Tahir Saeed Haroon, Tariq Mahmood, Ijaz Hussain
175
Chronic urticaria: frequency of anti-HCV antibodies
Ijaz Ahmed, Zarnaz Wahid, Zaffar Ahmed
179
Review articles
Histiocytoses
Mansoor Dilnawaz, Nasser Rashid Dar
184
10th Annual Conference of Dermatology. Abstracts.
193
Surgical pearl
Useful electrode modification for electrosurgery
Ahsan Hameed
202
Case reports
Neurofibromatosis type 1 with generalized pruritus
Arfan ul Bari, Humayun Agha, Simeen Ber Rahman
204
Atrophoderma vermiculata: A rare disfiguring condition
Arfan ul Bari
208
Quiz
What are these plaques?
Amor Khachemoune, Shahbaz A. Janjua
211
News
214
Author Index
215
Subject Index
217
Information for authors
221
Journal of Pakistan Association of Dermatologists 2003; 13: 161-163.
Editorial
Non-Langerhans cell histiocytoses
Farhana Muzaffar
Department of Dermatology, Institute of Child Health/Children Hospital, Lahore
Cutaneous histiocytic infiltrates can occur in
a number of conditions e.g. infections
(tuberculosis, leprosy, atypical mycobaterial
infections, leishmaniasis, subcutaneous
mycoses, etc.), trauma, foreign body
reactions (silica, glass, etc.), metabolic
(Gaucher’s
disease),
and
tumours
(Hodgkin’s disease, Lennert’s lymphoma)
etc. However, the term histiocytoses is
reserved for the idiopathic cases. According
to the Histiocyte Society,1 this group is
further divided into Langerhans cell (class
I), non-Langerhans cell (class II) and
malignant histiocytoses (class III) by their
clinical
features,
histopathological
characteristics and analysis of the
predominant cells. The non-Langerhans cell
histiocytoses (NLCH), also called nonhistiocytoses X, are a heterogeneous group
of frequently nonaggressive and self-healing
entities which affect both children and
adults. The clinical spectrum may range
from banal cutaneous involvement as in
dermatofibroma
to
severe
systemic
involvement as seen in hemophagocytic
syndrome.2 Histologically, these share the
common denominator of proliferation of
normal-looking histiocytes.3 The individual
cells may be spindle-shaped, scalloped,
Address for Correspondence
Dr. Farhana Muzaffar,
Assistant Professor of Dermatology,
Institute of Child Health/Children Hospital,
Lahore.
xanthomatized, vacuolated, or oncocytic.
Variable mixture of these cells, along with
Touton or foreign body giant cells, produce
different histopathological patterns which
may be monomorphous or polymorphous.
Ultrastructurally, these cells always lack
Birbeck (Langerhans) granules and are
usually S-100 and CD1a negative.3
Immunohistochemistry
reveals
an
inconsistent pattern of reactivity to various
antigens like S-100 protein; panmacrophage
antigens EBM 1, Leu-M3; antigens
functionally associated with phagocytosis
(Fc receptor for IgG, complement receptor
3); markers of lysosomal activity (lysozyme
1-antichymotrypsin, alpha 1-antitrypsin;
antigens associated with early inflammation
(Mac-37, 27E10); antigens commonly found
on monocytes but not tissue macrophages
(OKM5, Leu-M1 [CD 15]); activation
antigens (Ki-1 and receptors for transferin
and interleukin 2); and others e.g. CD31,
CD30, HM56.4 Gene rearrangement studies
reveal a polyclonal nature of infiltrate.5
The complex clinicopathological appearance
and the usually benign course of disease are
indicative of a reaction pattern rather than a
neoplastic process. The cause of this
remains unknown. Nonetheless, newer
studies reveal the role of superantigens in
immune mechanism stimulation after some
focal injury e.g. insect bite, folliculitis,
161
Non-Langerhans cell histiocytoses
Farhana Muzaffar
Figure 1 Unifying concept of non-Langerhans cell histiocytoses (NLH). Reaction pattern in different NLH entities
[8].
trauma or a systemic insult. Inappropriate
stimulation of macrophages, primary or
secondary to some infective agents e.g.
viruses (EBV, CMV, measles, etc.), bacteria
(Klebsiella, Brucella, etc.), parasites etc;
autoimmune or malignant process, leads to
phagocytosis of blood cells. A possible
pathogenic role of a defective function of
perforin, a protein involved in the cytolytic
processes and control of lymphocyte
proliferation, has been speculated and
162
mutations in perforin PRF1 gene have been
identified in a subset of patients with
hemophagocytic
lymphohistiocytosis.6
Inappropriate stimulation of macrophages in
bone marrow and lymphoid organs leads to
phagocytosis of blood cells and production
of proinflammatory cytokines especially
tumour necrosis factor-α (TNF-α).7 TNF-α
has been implicated as an important
cytokine in the process and anti-TNF-α
agents may prove an adjunctive therapy.8,9
Journal of Pakistan Association of Dermatologists 2003; 13: 161-163.
Attempts have been made to unify the
evolution of this diverse group. Under the
influence of different sets of cytokines, the
bone
marrow-derived
macrophagemonocyte cells develop into different cell
lines (Figure 1).8
As far as treatment is concerned, apart from
the excision of solitary lesions, it remains
unsatisfactory for most of the conditions.
Therapeutic studies are scanty due to the
rare nature of these conditions and usually
anecdotal reports are available.7-10 Some of
the conditions regress spontaneously, while
others may improve as the underlying
disease is treated. In secondary forms of
disease treatment is generally symptomatic
based on transfusion, correction of fluid and
electrolyte disturbances and etiological
therapies
(antivirals,
antibiotics,
immunosuppressives or chemotherapeutic
agents). Newer therapeutic options targeting
specific mediators including TNF-α may
emerge with better understanding of the
pathogenesis.
6.
Arico M, Allen M, Brusa S et al.
Haemophagocytic
lymphohistiocytosis.
Proposal of a diagnostic algorithm based on
perforin expression. Br J Hematol 2002;
119: 180-1.
7. Karras A, Hermine O. Hemophagocytic
syndrome. Rev Med Interne 2002; 23: 76878.
8. Zelger BWH. The histiocytoses. In: DyallSmith D, Marks R, eds. Dermatology at the
millennium. Proceedings of the 19th World
Congress
of
Dermatology.
London:
Parthenon Publishing Group. p. 343-7.
9. Ravelli A. Macrophage activation syndrome
(MAS). Curr Opin Rheumatol 2002; 14:
548-52.
10. Lay JD, Chuang SE, Rowe M, Su IJ.
Epstein-Barr virus latent membrane protein1 mediates upregulation of tumour necrosis
factor-alpha in EBV-infected T cells:
implications for the pathogenesis of
hemophagocytic syndrome. J Biomed Sci
2003; 10: 146-55.
References
1.
2.
3.
4.
5.
Writing Group of Histiocyte Society.
Histiocytic syndromes in children. Lancet
1987; i: 208-9.
Snow JL, Su WPD. Histiocytic diseases. J
Am Acad Dermatol 1995; 33: 111-23.
Burgdorf WHC. The histiocytoses. In: Elder
D, Elenitsas R, Jaworsky C, Johnson B Jr,
eds. Lever’s histopathology of the skin, 8th
edn. Philadelphia: Lippincott-Raven; 1997.
p. 591-616.
Eisen RN, Buckley PJ, Rosai J.
Immunophenotypic characteristics of sinus
histiocytosis with massive lymphadenopathy
(Rosai-Dorfman disease). Semin Diagn
Pathol 1990; 7: 74-82.
Paulli M, Bergamaschi G, Tonon L et al.
Evidence for a polyclonal nature of the cell
infiltrate in sinus histiocytosis with massive
lymphadenopathy (Rosai-Dorfman disease).
Br J Dermatol 2003; 91: 415-8.
163
Journal of Pakistan Association of Dermatologists 2003; 13: 164-170.
Original article
Childhood eczema: a comparative analysis
Ijaz Ahmed, Mujeeb Ansari, Kashef Malick
Baqai Institute of Skin Diseases, Baqai Medical University, Karachi.
Abstract Introduction Dermatological disorders affect children of all ages. Eczema is a common
problem, along with infections and infestations. This study was carried out to determine
frequency of various types of childhood eczema in our community.
Materials and methods The study comprises an audit of freshly registered cases of
childhood eczema presenting in the outpatient department of BISD from 1st October 2001,
till 31st March 2003, over a period of 18 months. A total of 1038 patients, of either sex, up to
15 years of age were included. There were 541 (52%) males and 497 (48%) females. All the
clinically diagnosed cases were subclassified into: atopic dermatitis, pityriasis alba,
seborrheic dermatitis, contact dermatitis, napkin dermatitis, xerotic eczema, infective eczema,
pompholyx, nummular eczema, and lichen simplex chronicus. The patients were divided into
three groups according to age; infancy, 1-5years and 5-15 years.
Results Atopic dermatitis (26%) was the most common type seen followed by pityriasis alba
(25%), seborrheic dermatitis (21%), contact dermatitis (7%), napkin dermatitis (7%), xerotic
eczema (6%), infective eczema (4%), nummular eczema (1.5%), pompholyx (1.5%) and
lichen simplex chronicus (1%). There were 445 (42.9%) infants, 283 (27.3%) in the 1-5 years
group and 309 (29.8%) in 5-15 years group. Atopic eczema (44%), seborrheic dermatitis
(65%), napkin dermatitis (88%) and xerotic eczema (51%) were the most common in infancy.
In the 1-5 year group, infective eczema (55%), atopic dermatitis (31%), pityriasis alba (28%)
and contact dermatitis (23%) were seen commonly. In the 5-15year group, pityriasis alba
(51%), contact dermatitis (47%), xerotic eczema (29.5%) and atopic dermatitis (24%) had the
highest frequencies.
Conclusion The clinical patterns of childhood eczema in our population are the same with
some variations in frequency. Most of the patients present within the first 5 years of life.
Key words
Childhood, dermatitis, eczema.
Introduction
Dermatological disorders affect children
of all ages. Eczema is a common problem,
although infections and infestations are a
major cause of dermatological ailments in
children.1 In neonates, eczematous
eruptions are transient and troublesome
but may herald chronic and severe atopic
Address for Correspondence
Dr. Ijaz Ahmad,
36/2, Khayaban-e-Shujaat, D-H-A, Phase 5,
Karachi.
Ph # 021-584842, 021-5848454
164
dermatitis or provide an important clue to
severe intrinsic diseases like zinc
deficiency and Leiner's disease.2 Infantile
eczema usually indicates underlying atopy.
In the later age, eczema reflects an
increasing exposure of children to weather
changes, wind, sunlight, environmental
pollution,
allergens
and
different
infectious agents.
Eczema is defined as an inflammatory
response of the skin to various endogenous
and
exogenous
factors,
clinically
Childhood eczema
Ijaz Ahmed et al.
characterized by itching, redness, scaling,
crusting, papulovesicular lesions and
lichenification, based on the clinical stage.
Depending upon the etiological factors
there can be endogenous or exogenous
types of eczema. Contact dermatitis and
infective eczema are examples of
exogenous eczema. Atopic dermatitis,
seborrheic dermatitis and pityriasis alba
belong to the group of endogenous
eczemas. Endogenous eczemas are more
commonly seen in patients with an atopic
background. Eczema is a disease of all age
groups, but certain types of eczema may
be more common at a particular age e.g.
asteatotic eczema in the elderly and atopic
dermatitis in children. Certain anatomical,
physiological, pathophysiological and
pharmacological
differences
are
responsible for this disease pattern in
juvenile and elderly subjects.3 Children up
to the age of 15 years constitute a major
proportion
of
our
population.4
Considerable ethnic variation occurs in the
prevalence of eczema in the general
population.5 Muzaffar et al.6 reported 25%
of children suffer from some form of
eczema.
both the sexes, up to 15 years of age were
included. There were 541 (52%) males and
496 (48%) females. After an appropriate
history, a cutaneous and systemic
examination was carried out. Any relevant
investigations e.g. scrapings for fungus,
swabs for culture and sensitivity, patch
test, biopsy and histopathology etc. were
performed as well. All the clinically
diagnosed cases were subclassified into
following types of eczema: atopic
dermatitis, pityriasis alba, seborrheic
dermatitis, contact dermatitis, napkin
dermatitis, xerotic eczema, infective
eczema, pompholyx, nummular eczema,
and lichen simplex chronicus. The patients
were also divided into 3 groups i.e. infants
(0-1 year), 1-5 years, and 5-15 years.
The current study was carried out to
determine the frequency of various types
of childhood eczema in our community
and to compare the results with any
previous study.4 Such a study can be
helpful in planning our health care system
in future.
Atopic dermatitis (26%) was the most
common type seen, followed by pityriasis
alba (25%), seborrheic dermatitis (21%),
contact dermatitis (7%), napkin dermatitis
(7%), xerotic eczema (6%), infective
eczema (4%), nummular eczema (1.5%),
pompholyx (l.5%) and lichen simplex
chronicus (1%). The frequencies of
different eczema were also studied in
accordance with age group. In the infantile
group 445 (42.9%) patients suffered from
eczema whereas 283 (27.3%) patients
were affected in the I-5 years group and
309 (29.8%) had the disease in the 5-15
Materials and methods
The study comprised an audit of the
freshly registered cases of childhood
eczema presenting in the outpatient
department of BISD, from 1st October
2001, till 31st March 2003, over a period
of 18 months. 1037 patients belonging to
Results
Of the total 1038 patients, there were 541
(52%) males and 497 (48%) females, the
male to female ratio being 1.1:1. More
females suffered from contact dermatitis,
nummular eczema and pompholyx. Males
predominated in all other types of eczema.
The relative frequencies of different types
of eczema are shown in Table 1.
165
Journal of Pakistan Association of Dermatologists 2003; 13: 164-170.
Table 1 Clinical patterns of childhood eczema (n =1038)
Sr. No.
Type of eczema
Male
Female
1.
2.
3.
4.
5.
6.
7.
8.
9.
10
Atopic dermatitis
Pityriasis alba
Seborrheic dermatitis
Contact dermatitis
Napkin dermatitis
Xerotic eczema
Infective eczema
Nummular eczema
Pompholyx
Lichen simplex chronicus
143
135
117
37
36
33
21
5
7
7
127
122
104
40
34
28
17
11
8
6
Total
n (%)
270 (26)
257 (25)
221 (21)
77 (7)
70 (7)
61 (6)
38 (4)
16 (1.5)
15 (1.5)
13 (1)
Table 2 Distribution of eczema in different age groups (n=1038)
Sr. No.
Type of eczema
1.
2.
3.
4.
5.
6.
7.
8.
9.
10
Atopic dermatitis
Pityriasis alba
Seborrheic dermatitis
Contact dermatitis
Napkin dermatitis
Xerotic eczema
Infective eczema
Nummular eczema
Pompholyx
Lichen simplex chronicus
Total
0-1 year
n (%)
120 (44)
53 (21)
144 (65)
23 (30)
62 (88)
31 (51)
10 (25.5)
2 (12.5)
445 (42.9)
years group. The frequency of atopic
dermatitis was 44% in infancy, followed
by 31% and 25% in the 1-5 and 5-15 years
groups, respectively (p<0.01). Pityriasis
alba (51%) was the most common variety
in the 5-15 years group followed by 28%
and 21% in the 1-5 and 0-1 year groups,
respectively
(p<0.001).
Descending
frequency of seborrheic dermatitis in
different age groups was infancy (65%), 15 years (25%) and 5-15 years group (10%)
(p<0.001). Contact dermatitis was most
commonly seen in the 5-15 year-old
patients (47%), followed by infancy (30%)
and 1-5 years (23%) (p< 0.01). Napkin
dermatitis had the highest presentation in
infancy (88%) and a lower frequency in
the 1-5 years group (12%) (p<0.05).
Xerotic eczema was seen with the
following frequencies: infancy (51%), 5-
166
1-5 year
n (%)
84 (31)
72 (28)
55 (25)
18 (23)
8 (12)
12 (19.5)
21 (55)
8 (50)
2 (13)
4 (31)
284 (27.3)
5-15 year
n (%)
66 (24)
132 (51)
22 (10)
36 (47)
18 (29.5)
7 (18.5)
6 (37.5)
13 (87)
9 (69)
309 (29.8)
Total
n
270
257
221
77
70
61
38
16
15
13
1038
15 years group (29%) and 1-5 years group
(20%) (p<0.01). Infective eczema
presented most commonly in the 1-5 years
group (55%), followed by infancy (26%)
and the 5-15 years group (19%) (p<0.05).
Nummular eczema, pompholyx, and
lichen simplex chronicus presented less
frequently in all age groups.
Discussion
The results of our study give an insight
into the frequency of different types of
childhood eczema. The most common
types of eczema were atopic dermatitis,
pityriasis alba and seborrheic dermatitis.
Other less frequently reported eczemas
included contact dermatitis, napkin
dermatitis, xerotic eczema and infective
eczema. Nummular eczema, pompholyx,
and lichen simplex chronicus were the less
Childhood eczema
common types seen. Males were affected
more commonly among all the patients but
females predominated in the following
types: contact dermatitis, nummular
eczema and pompholyx. The incidence of
eczema has been reported to be higher in
males previously. Childhood pyoderma is
a major dermatological problem in the
third world countries7 but Giam8 reported
eczema to be the most predominant group
of dermatoses in children, atopic
dermatitis being the most frequent type.
The frequency of eczema in our series
remained the highest during infancy (43%)
followed by 30% and 27% in the 5-15 and
1-5 years age groups, respectively.
Previous reports8,9 suggest eczema to be
the most prevalent in 1-5 years age group.
Muzaffar et al.4 also reported the highest
prevalence of eczema in 1-5 year-old
subjects (42.5%), a higher frequency in 515 years age group (31%) and a lower
figure in infancy (26.5%). Higher
frequency of atopic, seborrheic and napkin
dermatitis in our series during infancy may
contribute to this difference.
Atopic dermatitis is reported to be more
common in Asians and is especially seen
in urban areas.10 The prevalence of atopic
dermatitis is the highest in juvenile
subjects.11 It affects 10% of all infants.12 In
UK13 15% and in Germany14 11.3%
children suffer from the disease. Current
prevalence of atopic dermatitis in school
going children is 10-15.6%.15 Lawrence et
al.16 have reported an increased prevalence
of atopic dermatitis over the recent
decades in school going children. Atopic
dermatitis accounts for 10-15% of visits to
a pediatric dermatologist.17 It presented
with the highest frequency (26%) in our
series that is consistent with the previous
reports.8,9 The frequency is significantly
higher than that from Lahore (13.3%).4
This finding could be due to a hot humid
Ijaz Ahmed et al.
weather, environmental pollution and
higher extent of industrialization. The
male preponderance reported from Lahore4
and in the current series is in accordance
with the literature.10,18,19 Its significance
remains unclear. Most of the patients
presented in infancy (44%) and a total of
75% had presented within the first 5 years.
Report from Lahore4 also gives a
frequency of 75% for atopic dermatitis
within the first five years, but the ratio was
higher in 1-5 years old group (54%).
Almost 80% of the atopic dermatitis
subjects are reported to present by the age
of five years.20-22 Therefore, our findings
are consistent with the literature.
Pityriasis alba is a disease of children 3-16
years old.23 A disease well known to be
associated with atopy, but many cases may
lack an atopic background.4 The frequency
of pityriasis alba (25%) is higher than that
reported previously4 (17.5%). It is the
most common type of childhood eczema.
The disease involves both the sexes, but
males predominated in our study and also
in the past.4 The frequency of pityriasis
alba increases with age being the highest
in the 5-15 years age group (51%),
followed by 1-5 years group (28%) and
infancy (21%). This tendency with
increasing age is comparable with that
reported previously4 i.e. 5-15 years (46%),
1-5 years (41%) and infancy (13%).
Therefore, 80% of these subjects present
after infancy. The reason for this trend is
an increased exposure of a growing child
to wind, sunlight and detergents.
Seborrheic dermatitis is a multifactorial
disease predominantly affecting infants
and adults but can also involve children.
Seborrheic dermatitis is proposed to be a
characteristic pattern of atopic dermatitis
and not a distinct entity.24 It is the 3rd most
common type of childhood eczema in our
167
Journal of Pakistan Association of Dermatologists 2003; 13: 164-170.
series, its frequency (21%) being less than
that reported previously (26.2%).4 The
results differ from the reports of Giam et
al.8 (0.7%). Almost 90% of the patients
suffering from seborrheic dermatitis had
presented by the age of 5 years which is
comparable with data from Lahore4 (80%).
Infants (65%) had a frequency higher than
that from Lahore (38%).4 Malnourishment
and atopy may account for this high figure
in infancy.
Contact
dermatitis
is
a
disease
predominantly of adults but children are
not immune. The immune system is not
fully developed at birth. As the child
grows it matures and exposure to
environmental allergens increases, as well.
Therefore, irritant contact dermatitis is
more likely to be seen in infants but in the
older children allergic contact dermatitis
predominates.25 Common sensitizers are
not an infrequent culprit in children, as
indicated by positive patch tests.25-30 In our
series the frequency of contact dermatitis
(7%) was comparable to a past study from
Lahore (8.2%).4 However, more females
were affected in the latter.4 The age wise
distribution was also comparable with
30% patients presenting in infancy and
70% afterwards i.e. 1-15 years-old. An
inappropriate use of soaps and antiseptics
etc. accounts for 30% infants suffering
from contact dermatitis.
Napkin dermatitis, predominantly a
disease of infants, is an irritant type of
reaction involving the genital flexures.
Occlusive environment of the napkin area
combined with candidiasis, bacterial
infection and irritant effects of urine and
feces result in this condition. Napkin
dermatitis has a peak incidence in infancy
and affects 50% of the infants at some
stage.31 Napkin dermatitis (7%) had the
following frequency in different age
168
groups: infancy (88%) and 1-5 years
(12%). This frequency (7%) is higher than
that in the past (4.4%).4 However, the
disease was most commonly seen in
infancy as reported previously.4 It is
especially common in obese children.32
Xerotic eczema, seen less frequently in
children is usually a manifestation of
atopy but may be a presentation of
ichthyosis or malnutrition. The frequency
of xerotic eczema (6%) is even less than
half that reported previously (14.6%).4 Hot
and humid weather of Karachi, in contrast
to dry and cold weather in Lahore, may
account for this significant difference. In
our series half the children suffering from
xerotic eczema were infants, while 19.5%
were 1-5 year and 29.5% 5-15 year-old.
About 70% of the children presented by
the age of 5 years. This higher ratio in
infancy may reflect an atopic background.
Similar figures have been reported from
Lahore,4 65% patients presenting within
the first 5 years, although the ratio was
higher in 1-5 years age group (44%).
Infective eczema is an inflammatory
reaction of the skin to different
microorganisms e.g. bacteria, viruses and
fungi. The frequency (4%) is almost onethird of that reported in the past (13.3%).4
In the current series, the highest frequency
was seen in the 1-5 year-old subjects
(55%), which is almost comparable to
Lahore (46%).4 The other age groups had
the following frequencies: infancy (26%)
and 5-15 yr. (18.5%). The comparative
figures in the past study4 were: 20% in
infancy and 34% in 5-15 year group. Thus
most of the patients in either series
presented by the age of 5 years.
Nummular eczema (1.5%), pompholyx
(1.5%), and lichen simplex chronicus (1%)
Childhood eczema
were least frequent and in accordance with
previous study.4
Conclusion
Similar clinical patterns of childhood
eczema are seen in our population with
some variation in frequency. Atopic
dermatitis, seborrheic dermatitis and
pityriasis alba remain the most common
types. Most of the patients present within
first 5 years of life. Atopic dermatitis
predominates in Karachi.
References
1.
Sharon S. Raimer. New and emerging
therapeutics in pediatric dermatology.
Dermatol Clin 2000; 18: 73-8.
2. Eady RAJ, Leigh IM, Pope FM. Anatomy
and organization of human skin. In:
Champion RH. Burton JL, Burns DA,
Breathnach SM. Rook/Wilkinson/Ebling
textbook of dermatology, 6th edn. London:
Blackwell Science; 1998. p. 37-111.
3. Khan Y. Neonatal dermatology. J Pak
Assoc Dermatol 2000; 10: 1-2.
4. Muzaffar F, Hussain I, Rashid T. An audit
of childhood eczema. J Pak Assoc
Dermatol 2000; 10: 9-14.
5. Boukner NC, Cross KW, Well M.
Sociological
implications
of
an
epidemiological study of eczema in the
city of Birmingham. Br J Dermatol 1976;
95: 135-44.
6. Muzaffar F, Hussain I. Pattern of skin
diseases at Children Hospital, Lahore. J
Pak Assoc Dermatol 2000; 10: 21-5.
7. Qureshi AA, Qureshi AS, Shah SA.
Vitiligo. J Pak Assoc Dermatol 1992; 2: 116.
8. Giam YC. Skin diseases in children in
Singapore. Ann Acad Med Singapore
1988; 17: 569-72.
9. Goh CL, Akarpanth R. Epidemiology of
skin diseases among children in a referral
clinic in Singapore. Pediatr Dermatol
1994; 11: 125-8.
10. Hanifin J. Atopic dermatitis in infants and
children. Pediatr Dermatol l991; 38: 76391.
11. Muzaffar F, Hussain I, Rani Z et al.
Emollients as an adjunct to topical
corticosteroids in children with mild to
moderate atopic dermatitis. J Pak Assoc
Dermatol 2002; 12: 64-8.
Ijaz Ahmed et al.
12. Hanifin J, Schnieder-Donald Y, Leung J,
Howard
S.
Recombinant
gamma
interferon therapy of atopic dermatitis. J
Am Acad Dermatol l993; 28: 189-97.
13. Cox HE. Moffat PM, Faux JA, Walley AI.
Association of atopic dermatitis to the beta
subunit of high affinity IgE receptors. Br J
Dermatol 1998; 138: 182-7.
14. Schafer T, Dockery A, Kramer U.
Experience with the severity score of
atopic dermatitis in a population of
German preschool children. Br J Dermatol
l997; 137: 558-62.
15. Wuthrich
B.
Clinical
aspects,
epidemiology and prognosis of atopic
dermatitis. Ann All Asth Immunol 1999;
83: 464-70.
16. Lawrence F, Lucky MD, Richard G,
Langley B. Safety and efficacy of
pimecrolimus cream 1% in the treatment
of mild to moderate atopic dermatitis in
children and adolescence. J Am Acad
Dermatol 2002; 40: 495-507.
17. Hanifin JM, Saurat JR. Underlying atopic
dermatitis: pathophysiology and etiology
in children. J Am Acad Dermatol 2001;
45: S1-S8.
18. Dhar S, Kanwar AI. Epidemiological and
clinical pattern of atopic dermatitis in a
North
Indian
population.
Pediatr
Dermatol l998; 15: 347-51.
19. Rajika G, ed. Essential aspects of atopic
dermatitis, 1st edn. Berlin: SpringerVerlag; 1989.
20. Sidburg R, Ion M, Hanifin JM. Old, new
and emerging therapies for atopic
dermatitis. Dermatol Clin 2000; 18: 1-11.
21. Kay O, Gawkrodger FM, Mortimer MJ et
al. The prevalence of atopic dermatitis in
general population. J Am Acad Dermatol
1994; 30: 35-9.
22. Hanifin JM, Chan SC. Diagnosis and
treatment of atopic dermatitis. Dermatol
Ther 1996; 1: 9-18.
23. Zaynoun ST, Aftimas BG, Tenejian KK.
Extensive
pityriasis
alba,
a
histopathological and ultrastructural study.
Br J Dermatol 1983; 108: 83-90.
24. Vickers CFH. The natural history of
atopic eczema. Acta Derm Venereol 1980;
93: 113-5.
25. Gulliet MH, Cartier H, Gulliet G.
Evaluation of contact dermatitis in
childhood, cross sectional evaluation in
152 children. J Eur Acad Dermatol l997;
7: 56-8.
26. Hjorth N. Contact dermatitis in children.
Acta Derm Venereol Suppl Stockh 1981;
95: 36-9.
169
Journal of Pakistan Association of Dermatologists 2003; 13: 164-170.
27. Weismann K, Krakaner R, Wanscher B.
Prevalence of skin diseases in old age.
Acta Derm Venereol 1980; 60: 352-3.
28. Coenrads PJ, Nater JP, Vanderlande R.
Prevalence of eczema and other
dermatoses of the hands in The
Netherlands. Clin Exp Dermatol 1983; 6:
495-503.
29. Balato N, Lembo G, Patrumo C et al.
Patch testing in childhood. Contact
Dermatitis 1989; 20: 305-6.
30. Stabley GI, Forsyth A, Lener RS. Patch
testing in childhood. Contact Dermatitis
1996; 34: 341-4.
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31. Jordan WE, Lawson KD, Berg RW.
Diaper dermatitis: frequency and severity
among a general population. Pediatr
Dermatol l986; 43: 198-207.
32. Atherton DJ. The neonate. In: Champion
RH, Burton JL, Burns DA, Breathnach
SM, eds. Rook/Wilkinson/Ebling textbook
of Dermatology, 6th edn. London:
Blackwell Science; 1998. p. 449-518.
Journal of Pakistan Association of Dermatologists 2003; 13: 171-174.
Original article
Role of serodiagnosis in cutaneous leishmaniasis
Simeen ber Rahman, Arfan ul Bari,* Haroon ur Rashid†
Military Hospital, Rawalpindi.
* PAF Hospital, Sargodha.
† Army Medical college, Rawalpindi.
Abstract Background Cutaneous leishmaniasis caused by different species of Leishmania parasite, is
endemic in various regions of Pakistan. It is probably the second most prevalent vector-borne
disease in the country (after malaria). Diagnosis is mostly made by its clinical presentation,
especially in the endemic areas. Sometimes it is aided by slit skin smear examination,
histopathological study and parasite culture. Considering the fact that serology has made
significant advances in diagnosing various parasitological diseases, this study was carried out
to evaluate the role of serological techniques in diagnosis of cutaneous leishmaniasis.
Patients and methods Three serological tests i.e. enzyme-linked immunosorbent assay,
immunofluorescent antibody test, indirect hemagglutination test were done in 57 clinically
diagnosed cases of cutaneous leishmaniasis.
Results Positive results were seen in 62.4%, 52% and 52% with ELISA, IFAT, and IHA tests
respectively.
Conclusion Serological tests can be used as a supporting and screening investigation but not
to make the final diagnosis.
Key words
Cutaneous leishmaniasis, serological tests, enzyme-linked-immunosorbent assay (ELISA),
immunofluorescent antibody test (IFAT), indirect hemagglutination test (IHT)
Introduction
Leishmaniasis comprises a heterogeneous
spectrum of diseases caused by different
species of a protozoan parasite
Leishmania. The spectrum of the disease
ranges between a self-healing cutaneous to
a more progressive life threatening
systemic condition.1,2 Leishmaniasis is
endemic in many parts of the world
including Pakistan. Cutaneous variant of
the disease is seen here although there are
pockets of the visceral form in the
northern part of the country.3,4 In view of
Address for Correspondence
Squadron Leader Dr. Arfan ul Bari,
Consultant Dermatologist,
PAF Hospital, Sargodha.
E mail: albariul@yahoo.com
the large number of clinical presentations
of cutaneous leishmaniasis (CL), a correct
diagnosis of the disease becomes
important. The clinical picture may be
distorted by superadded infections or
badly managed treatment and these may
cause great difficulty in diagnosis.3,5
Moreover, conventional methods of
laboratory diagnosis have also certain
limitations.6,7 In this scenario various
immunodiagnostic techniques do help in
establishing an accurate diagnosis.
Leishmanial parasite contains some
antigenic components that evoke both
humoral as well as cell-mediated immune
responses.8 The cell-mediated immune
response can be demonstrated by delayed
hypersensitivity
reactions
and
171
Role of serodiagnosis in cutaneous leishmaniasis
histopathological picture and to detect
humoral response various serological tests
have been developed which help in the
diagnosis of CL. These include
complement fixation test (CFT), indirect
hemagglutination test (IHT), direct
agglutination test (DAT), gel precipitin
test (GPT), gel diffusion and counter
current
electrophoresis
(CEP),
immunofluorescent antibody test (IFAT),
enzyme linked immunosorbent assay
(ELISA),
peroxidase
antiperoxidase
methods
(PAP)
and
monoclonal
antibodies.8-11 This study was focused only
to see the humoral response and was
aimed to evaluate and compare the
effectiveness of some commonly used
serological tests namely ELISA, IFAT and
PAP.
Materials and methods
Fifty-seven patients of both sexes,
belonging to all age groups and having
clinically suggestive lesions of cutaneous
leishmaniasis, were selected on the basis
of following criteria: (i) All these patients
were from the known endemic areas or
had visited the endemic areas during last
six months. (ii) All had skin lesions in the
form of either discrete nodules or had nonhealing ulcers for the past few weeks. (iii)
They had not received treatment with
pentavalent
antimonial
compounds
(sodium stibogluconate or meglumine
antimonate). (iv) Few of these clinically
suspected patients had positive skin slit
smears for Leishman-Donovan (LD)
bodies. All negative skin slit smears were
also included (because negative smear
does not rule out the diagnosis of CL).
Nine patients were excluded because of
the following reasons: (i) Five patients had
skin lesions that appeared like the infected
lesions of cutaneous leishmaniasis but
resolved after a short course of antibiotics.
(ii) Three refused to give blood for
172
Simeen ber Rahman et al.
serological tests. (iii) One sample was
omitted as it turned out to be a case of
deep mycosis. Sera of all patients were
screened with three different methods to
see the presence and the levels of
antibodies in the sera of the clinically
positive cases. The negative and the
positive controls were the same for each
test. Different sera dilutions were used and
following three methods were used for
serological analysis; (a) enzyme-linked
immunosorbent assay (ELISA). The
dilution used was 1/2. (b) Indirect
fluorescent antibody test (IFAT). The sera
dilutions used were 1/4 -- 1/8 ----- 1/256.
The dilutions below 1/8 were considered
negative, up to 1/16 were low levels and
up to 1/256 were high levels. (c) Indirect
hemagglutination
(IHA)
test.
The
commercial kit used for this method was
for Leishmania donovani. The data was
recorded and analyzed in Microsoft Excel
programme using frequencies and
percentages.
Results
Out of 48 patients, 28 were from
Baluchistan (25 males and 3 females) and
their ages ranged from 7-43 years. The 20
patients reporting directly to Military
Hospital, Rawalpindi were; 9 from
different parts of northern areas, 6 from
Chakwal area and 5 from Kohat (13 males,
3 females, 4 children) and their ages
ranged from 6 months to 35 years. The
duration of illness varied between 3-18
months. Clinically the lesions were
basically of three types; (i) Wet type (early
ulcerative, rural) in 34 patients. (ii) Dry
type (late ulcerative, urban) in 15 and (iii)
Chronic cutaneous type in 1 patient.
Serology was positive in 29 patients with
ELISA technique (clinically they were 20
Journal of Pakistan Association of Dermatologists 2003; 13: 171-174.
Table 1 Comparative results of various
serological tests (n=48)
Sr.
No.
Serological tests
1
Enzyme-linked
immunosorbent assay
Indirect fluorescent
antibody test
Indirect
hemagglutination test
2
3
No. of
positive
cases (%)
29 (60.4)
25 (52.0)
25 (52.0)
wet type, 8 dry and 1 chronic type). IFAT was
positive in 25 patients (clinically they were 17
wet type, 7 dry and 1 chronic). IHA also
yielded 25 positive results (18 wet type, 6 dry
and 1 chronic) [Table 1].
Discussion
For establishing a diagnosis of CL, clinical
appearance of the lesions and history of
visit to an endemic area are usually
sufficient.5 These days the diagnosis is
generally aided by slit skin smear
examination,
impression
smears,
histopathology of the tissue sections and
by culture of parasite (where facilities are
available).6,7 However, unusual clinical
presentation, superadded infection or
persistence of the disease for a prolonged
time, has made scientists to develop more
sensitive methods to detect the disease.3
Moreover the conventional diagnostic
methods have their own limitations; slit
skin smears or impression smears give
good results only when the lesions are not
secondarily infected and skin biopsies may
give inconclusive results.6,7 An appropriate
screening test was always required for CL.
Our study was basically aimed to evaluate
the efficacy of serological tests in
diagnosis of CL and to compare various
available serological tests and no such
study was previously done in Pakistan.
Patients in our study represented a
heterogeneous population group and
almost all of them came from the areas
where the terrain is mostly mountainous or
hilly. The obvious common factor
appeared to be geographical distribution of
endemic areas.1 Clinical profiles of the
patients were almost similar as seen in
previous studies.1,12 A significantly low
percentage of females in this series was
due to the fact that the study was primarily
done on serving soldiers. The results of
serology by three different methods were
significant and ELISA was found slightly
superior to the other two. These
serological techniques have a supportive
role in diagnosis of CL, but these alone
cannot be taken as diagnostic, because
serology may remain positive for a long
time. Another drawback is that false
positive results may be seen with these
methods. It may however be used in
screening out clinically suspected cases in
largely endemic areas or in an epidemic.8-11
Conclusion
Serological tests are a good addition to
diagnostic armamentarium of CL. These
can be used to support the diagnosis and
for screening purposes in endemic areas
but should not be used alone to make final
diagnosis.
References
1.
2.
3.
4.
5.
Grevelink SA, Lerner EA. Leishmaniasis.
J Am Acad Dermatol 1996; 34: 257-72.
Lainson R, Shaw JJ. Evolution,
classification,
and
geographical
distribution. In: Peters W, KillickKendrick R, eds. The leishmaniasis in
biology and medicine. San Diego:
Academic Press; 1987. p. 1-120.
Raja KM, Khan AA, Hameed A, Rahman
SB. Unusual clinical variants of cutaneous
leishmaniasis in Pakistan. Br J Dermatol
1998; 139: 111-3.
Mujtaba G, Khalid M. Cutaneous
leishmaniasis in Multan, Pakistan. Int J
Dermatol 1998; 37: 843-6.
Bergan RS. Leishmania, touch preparation
as a rapid mean of diagnosis. J Am Acad
Dermatol 1986; 16: 1096-1105.
173
Role of serodiagnosis in cutaneous leishmaniasis
6.
Ridley DS. The laboratory diagnosis of
tropical diseases: Review. J Clin Pathol
1974; 27: 435-44.
7. Sharquie KE, Hassen AS, Hassan SA, AlHamami IA. Evaluation of diagnosis of
cutaneous leishmaniasis by direct smear,
culture and histopathology. Saudi Med J
2002; 23: 925-8.
8. Manuel J, Behin R, eds. Immunology of
parasitic infections, 2nd edn. Sidney:
Cotton and Kenneth Swarren; 1982.
9. Voller A, Bidweil D. Antigen detection by
Elisa. Asean J Clin Sci 1985; 5; 121-3.
10. Follador I, Araujo C, Bacellar O et al.
Epidemiologic and immunologic findings
174
Simeen ber Rahman et al.
for the subclinical form of Leishmania
braziliensis infection. Clin Infect Dis
2002; 34: 54-8.
11. Ryan JR, Smithyman AM, Rajasekariah
GH et al. Enzyme-linked immunosorbent
assay based on soluble promastigote
antigen detects immunoglobulin M (IgM)
and IgG antibodies in sera from cases of
visceral and cutaneous leishmaniasis. J
Clin Microbiol 2002; 40: 1037-43.
12. Ridley DS. A histological classification of
cutaneous
leishmaniasis
and
its
geographical expression. Trans R Soc
Trop Med Hyg 1980; 74: 515-9.
Journal of Pakistan Association of Dermatologists 2003; 13: 175-178.
Original article
Evaluation of sensitivity of Tzanck smear in
pemphigus
Jameel A. Shaheen, Tahir Saeed Haroon,* Tariq Mahmood,* Ijaz Hussain*
Department of Dermatology, Quaid-e-Azam Medical College/Bahawalpur Victoria Hospital
Bahawalpur
* Department of Dermatology, King Edward Medical College/Mayo Hospital Lahore.
Abstract Background Pemphigus is a group of immune-mediated, acantholytic disorders. Although
direct immunofluorescence is the gold standard for the diagnosis of this group of diseases, it
is not widely available and cost-effective in our country. Tzanck smear is a simple and easy
test used to demonstrate acantholytic cells from the lesions of disease. The present study was
planned to evaluate the sensitivity of Tzanck smear, in the diagnosis of pemphigus group of
diseases.
Patients and methods Tzanck smears were prepared from 37 cases of active pemphigus (17
males and 20 females, age range 11-65 years), diagnosed by clinical, histopathological and
direct immunofluorescence data. Patterns and positivity rate of Tzanck smears were recorded.
Results Overall sensitivity of Tzanck smears was 73%. Smears were positive in 75% of
cutaneous lesions and 69% of mucosal lesions (p>0.05). Acantholytic cells were seen
individually in all positive cases (100%), clumps (81%) and sheets (70%).
Conclusion Tzanck smears can be used as first-line investigation in our scenario. More
sophisticated investigation e.g. direct immunofluorescence may be reserved for Tzanck–
negative cases.
Key words
Tzanck smear, pemphigus, sensitivity
Introduction
Pemphigus is a group of immunemediated, intraepithelial acantholytic
blistering diseases, involving the skin and
mucous membranes. Amongst the
immunobullous disorders, it is the most
common entity.1 The disease is further
divided into three types i.e. pemphigus
vulgaris, pemphigus foliaceous, and
paraneoplastic pemphigus, with regard to
their distribution, morphological features,
histopathologic and immunofluorescence
findings, antigenic specifications, and
Address for Correspondence
Dr. Jameel A. Shaheen,
Assistant Professor of Dermatology,
Quaid-e-Azam Medical College/Bahawal Pur,
Victoria Hospital, Bahawalpur.
E mail: jshaheen@bwp.wol.net.pk
associated
conditions.2
Direct
immunofluorescence is an essential
investigation for the diagnosis of all types
of pemphigus with high degree of
sensitivity and specificity.1,2 However, the
technique is expensive to install and
maintain. Due to the same reasons this
technology is available at only few centres
in Pakistan.
Tzanck, in 1947, pioneered the work on
cutaneous cytology and evolved a method
for the cytological diagnosis of various
blistering diseases.3 Tzanck smears are
still widely used for the diagnosis of
175
Evaluation of sensitivity of Tzanck smear in pemphigus
acantholytic bullous diseases and herpes
viral infections. This is a relatively simple
and easy-to-perform technique. The
present study was planned to evaluate the
sensitivity of Tzanck smears in pemphigus
in
relation
to
the
direct
immunofluorescence, the gold standard
investigation for this disease.
Patients and methods
This study was conducted at the
Department of Dermatology, King Edward
Medical College/Mayo Hospital, Lahore.
Thirty seven cases of active pemphigus
(17 males and 20 females, age range from
11 to 65 years), were included in the
study. Clinical and investigative data were
recorded on a specially designed pro
forma. The diagnosis was confirmed by
performing direct immunofluorescence on
perilesional
skin/mucosal
biopsy
specimens.
Histopathology
(with
hematoxylin and eosin staining) on
lesional skin and mucous membrane was
performed to determine the subtype of
pemphigus. Tzanck smears were prepared
from cutaneous bullae (24 cases) or
mucosal ulcers (13 cases) for Giemsa
staining.
For Tzanck smears, a fresh, non-infected,
unruptured bulla was selected. Lesions
were wiped with alcohol and allowed to
dry for one minute. After deroofing the
blister, its floor was scraped with the edge
of a scalpel blade. The material obtained
was gently transferred from the blade to
the glass slide to make a smear. In case of
mucosal involvement similar smears were
prepared from the floor of the erosions.
For staining, the smears were flooded with
diluted stain for 15 minutes. The excess
stain was rinsed out and slides were
allowed to dry. The stained smears were
176
Jamil A. Shaheen et al.
examined under light microscope for the
presence of acantholytic cells.
For direct immunofluorescence study,
perilesional skin/mucosal biopsies were
wrapped in a gauze piece soaked with
normal saline. These specimens were
frozen and stored in liquid nitrogen
immediately.
For
sectioning,
the
specimens were embedded in embedding
medium, and cut at about -30ºC to a
thickness of 5 microns and placed on a
glass slide. Five sections per slide and 2-3
slides per patient were prepared. The
sections were allowed to air-dry for about
10 minutes. For DIF staining of these
biopsy sections, the slides along with
positive and negative controls were rinsed
in phosphate-buffered saline (PBS)
(pH=7.2). The sections were overlaid with
fluorescein
isothiocyanate
(FITC)
conjugated antisera i.e. antihuman IgG,
IgA, IgM, and C3 (The Anstar [Ltd],
USA). The slides were incubated in a
moist and closed plastic container for 20
minutes, followed by three washes of
fifteen minutes each in PBS to remove the
unreacted antiserum. They were allowed
to drain, and the excess fluid was wiped
off with dry cotton gauze. The slides were
mounted using a drop of buffered glycerin
as the mounting medium (90% glycerin in
PBS). Finally, the specimens were
examined under a fluorescence ultraviolet
microscope for their immunofluorescence
patterns.
Chi-square test was used for statistical
evaluation.
Results
Of 37 cases, 31 (83.8%) had pemphigus
vulgaris, 5 (13.5%) pemphigus foliaceus,
and one (2.7%) pemphigus vegetans.
Journal of Pakistan Association of Dermatologists 2003; 13: 175-178.
Figure 1 Tzanck smear with Giemsa staining showing a sheet and individual acantholytic cells.
Mean duration of the disease was 26
months ranging from three weeks to eight
years.
Direct
immunofluorescence
showed
deposition
of
immunoreactants
at
intercellular spaces (ICS) of epidermis in
all (100%) patients. The most common
immunoreactant detected was IgG, singly
or in combinations, in all patients (100%)
followed by C3 in 13 patients (35%), IgM
in 2 patients (5.4%), and with IgA in one
patient (2.7%).
On histopathological examination 15
patients were diagnosed as pemphigus
vulgaris, 4 as pemphigus foliaceus and 1
as pemphigus vegetans. Histopathological
features in 6 patients were nonspecific and
their
diagnosis
was
based
on
immunofluorescence findings and clinical
features. Tzanck smears were prepared
from cutaneous and mucosal lesions in 24
and 13 patients, respectively. Overall 27
patients (73%) showed positive results i.e.
acantholytic cells, significantly lower as
compared to DIF (p<0.05). The majority
showed more than one morphological
patterns. The cells in positive cases were
individually scattered (100%) or in the
form of clumps (81%) and sheets (70%)
[Figure 1]. Positivity rate among the oral
lesions was 69% as compared to 75% in
skin lesions (p<0.05) [Table 1].
Morphological patterns in mucosal smears
were identical to that of skin smears.
Discussion
Tzanck smears sensitivity was only 73%
as compared to DIF, a less sensitive test
but with certain advantages. Smears are
easy to make and less traumatic as
compared to skin or mucosal biopsies.
There is no need to preserve the specimens
in transport medium or to section them.
By-passing these two important steps
makes the test economical, time saving
and technically less demanding. The
technique of preparing the Tzanck smears
has been established as an easy, early and
177
Evaluation of sensitivity of Tzanck smear in pemphigus
rapid aid to the diagnosis of pemphigus as
described previously.4,5
Presently, there is a general agreement that
direct immunofluorescence staining at ICS
in epidermis is invariably positive in case
of active pemphigus, the diagnosis of
which should be seriously questioned if
this is found negative.2 However, this
technique has certain limitations especially
in countries with poor resources. It is
expensive to install (cryocut, ultraviolet
fluorescence microscope) and maintain
e.g. fluorescein-labelled antisera, liquid
nitrogen, different solutions etc. A special
biopsy is required from the perilesional
area, further traumatizing the patient.
Cryostat sectioning, labelling, and
interpretation of slides require much more
expertise as compared to Tzanck smears.
Tzanck smears, in our study, were
negative in about one-fourth of cases.
Different reasons for negative results may
be the procedure limitations adopted for
obtaining the cells on glass slides,
inflammatory infiltrate destroying the
antibody-labelled targeted cells, or partial
treatment with glucocorticoids. In
previous studies on the subject, Decherd et
al.6 performed the test on only two patients
and reported positive results. Though the
results were 100% but the total number of
cases was very low.
Patterns of cells in Tzanck smears were in
the form of individually scattered cells,
clumps or sheets, identical from both oral
and cutaneous lesions. This might be
dependent on the severity of inflammatory
reaction. Similarly, the positivity was
similar in oral or cutaneous lesions (69%
vs. 75%, p>0.05). No significant
correlation of positivity of smears with
age, sex or duration of the disease was
detected.
178
Jamil A. Shaheen et al.
Table 1 Sensitivity of Tzanck smear from
cutaneous and oral lesions
Skin lesions
Oral lesions
(n=24)
(n=13)
Positive
18 (75%)
9 (69%)
Negative
6 (25%)
4 (31%)
Concluding, though the Tzanck smear is
less sensitive as compared to DIF on
biopsy specimens, the simplicity of the
test is a considerable edge. Keeping the
sensitivity and simplicity together, the test
may be used for screening pemphigus
patients. Biopsy may be indicated in
negative cases. The test may be especially
helpful in situations where biopsy is
refused or is difficult to perform.
Collaboration of the test with clinical and
histological parameters can further
sharpen the diagnosis of pemphigus.
References
1. Korman N.
Pemphigus. J Am Acad
Dermatol 1988; 18:1219-38.
2. Stanley JR. Pemphigus. Freedberg IM,
Eisen AZ, wolff K et al., eds. Fitzpatrick’s
dermatology in general medicine, 5th edn.
New York: McGraw-Hill; 1999. p. 65465.
3. Ruocco V, Ruocco E. Tzanck smear, an old
test for the new millennium; when and
how? Int J Dermatol 1999; 38: 830-4.
4. Skeete MVH. Evaluation of the usefulness
of immunofluorescence on Tzanck smears
in pemphigus as an aid to diagnosis. J Clin
Exp Dermatol 1977; 2: 51-63.
5. Verma KK, Khaitan BK, Singh MK.
Antibody deposits in Tzanck smears in
pemphigus vulgaris. J Cutan Pathol 1993;
20: 317-9.
6. Decherd JW, Rubin MB, Goldschmidt H,
Heiss HB. Immunofluorescence of Tzanck
smears in pemphigus vulgaris. Acta Derm
Venereol (Stockh) 1972; 52: 116-8.
Journal of Pakistan Association of Dermatologists 2003; 13: 179-183.
Original article
Chronic urticaria: frequency of anti-HCV antibodies
Ijaz Ahmed, Zarnaz Wahid,‫ ٭‬Zaffar Ahmed
Baqai Institute of Skin Diseases, Baqai Medical University, Karachi.
‫٭‬Dow Medical College/Civil Hospital, Karachi.
Abstract Background Urticaria is defined as a skin disorder, clinically characterized by the presence of
transient erythematous itchy weals presenting as an acute or a chronic disease, which may be caused
by a wide variety of factors. Among infectious causes, hepatitis B virus is a known cause of
urticaria; it would not be surprising if hepatitis C virus turns out to be an important one as well. The
current study was aimed to determine the frequency of anti-BCV antibodies and HCV RNA in
patients with chronic urticaria and to study HCV as a cause of urticaria.
Patients and methods Of all the clinically diagnosed cases of chronic urticaria presenting in the
outpatient department of Baqai Institute of Skin Diseases, patients fulfilling the inclusion criteria
were enrolled in the study from 1st January, 2002 till 30th June, 2003 over a period of eighteen
months. Patients belonging to either sex were aged 11 to 55 years. A written consent was taken from
all the enrolled patients to carry out various blood tests. Sera of all the enrolled patients were tested
for anti-HCV antibodies. Patients with positive enzyme-linked immunosorbent assay were also
tested for viral RNA in their blood (polymerase chain reaction). Other relevant investigations were
also carried out in seropositive patients i.e. liver function tests and ultrasound abdomen.
Results Of the 114 patients enrolled in the study, 51 (44.8%) were males and 63 (55.2%) females,
the gender ratio being 0.8. The maximum age of presentation was 55 years while minimum was 11
years. Maximum number of patients was 21-30 years of age (42.1%). Fifteen patients (13.2%) were
positive for anti-HCV antibodies, comprising 9 females (7.8%) and 6 (5.6%) males (p<0.003).
Maximum number of these patients (7.1%) was aged 21-30 years. Five (4.4%) of these seropositive
subjects were also positive for HCV RNA including three males (2.6%) and two (1.8%) females.
Deranged liver function tests were a feature in only 2 of these patients.
Conclusion There may be a significant association of chronic urticaria with anti-HCV antibodies.
Further studies are necessary to establish or refute an aetiopathogenetic role of HCV in this
condition.
Key words
Hepatitis C virus, hepatitis B virus, urticaria, anti-HCV antibodies, seropositive
Introduction
Urticaria is defined as a skin disorder, clinically
Address for Correspondence
Dr. Ijaz Ahmad,
36/2, Khayaban-e-Shujaat, D-H-A, Phase 5,
Karachi.
Ph # 021-584842, 021-5848454
characterized by the presence of transient
erythematous itchy weals presenting as an acute
or a chronic disease, which may be caused by a
wide variety of factors. Among infectious
causes, hepatitis B virus (HBV) is a known
cause of chronic urticaria; it would not be
surprising if hepatitis C virus (HCV) turns out to
179
Chronic Urticaria: frequency of anti-HCV antibodies
be an important one as well.1 However, the
association of chronic urticaria and hepatitis C
remains tenuous. An association of chronic
urticaria with hepatitis "C" was initially based
on case reports.2 Numerous extrahepatic
disorders have been recognized in association
with
HCV
infection
among
which
dermatological diseases occupy a central place.
Cutaneous necrotizing vasculitis, mixed
cryoglobulinemia, porphyria cutanea tarda and
lichen planus are the major skin diseases
frequently associated with HCV infection, but
other skin disorders, such as Behcet syndrome,
erythema
multiforme
and
nodosum,
malakoplakia, urticaria and pruritus may also be
linked to hepatitis C.3 Recent investigations
suggest that two cutaneous diseases, lichen
planus and porphyria cutanea tarda, may signal
the presence of HCV infection.4,5 European trials
noted much lower rates of infection in patients
with chronic urticaria.6-8 Regarding dermatoses
e.g., in lichen planus, the frequency of anti-HCV
antibodies is 23.5% in our country;9 however,
the frequency of anti-HCV antibodies in chronic
urticaria has not been studied previously. The
current study was aimed to determine the
frequency of anti-HCV antibodies and "HCV
RNA" in patients with chronic urticaria and to
study HCV as a cause of urticaria.
Patients and methods
Of all the clinically diagnosed cases of chronic
urticaria presenting in the outpatient department
of Baqai Institute of Skin Diseases (BISD),
patients fulfilling the inclusion criteria were
enrolled in the study from 1st January, 2002 till
30th June, 2003 over a period of eighteen
months. Detailed history and clinical
examination comprising skin, general and
systemic examination was carried out and the
findings were recorded on a preformed pro
forma. A history of jaundice, blood transfusion,
medical, surgical and dental procedures was also
180
Ijaz Ahmed et al.
taken. Patients belonging to both sexes were
aged 11 to 55 years. Patients suffering from
chronic urticaria not responding to conventional
therapy for a period of 6 weeks were enrolled.
Patients with a history of jaundice, hepatitis,
impaired liver function tests or a history of
hepatotoxic drug intake were ruled out from the
study. Patients with a history of urticarial drug
eruption or any other evident cause of urticaria
were also excluded. A written consent was taken
from all these patients to carry out various
investigations. Sera of all the enrolled patients
were tested for anti-HCV antibodies. The
enrolled patients with positive enzyme-linked
immunosorbent assay (ELSA) were also tested
for viral RNA in their blood (polymerase chain
reaction). Other relevant investigations were
also carried out in seropositive patients i.e. liver
function tests and ultrasound abdomen. Liver
biopsy was not performed in any of the subjects.
Results
Of the 114 patients enrolled in the study,
51(44.8%) were males and 63 (55.2%) females,
the gender ratio being 0.8. The maximum age of
presentation was 55 years while minimum was
11 years. The mean age of presentation was 34.8
years. The mean ages for males and females
were 37.8 and 37.1 years, respectively. Table 1
reveals that the maximum number of patients
presented were 21-30 years of age (42.1%).
Table 2 shows that fifteen patients (13.2%) were
positive for anti-HCV antibodies, comprising 9
females (7.8%) and 6 (5.6%) males (p<0.003).
Maximum numbers of these patients (7.1%)
were aged 21-30 years. Five (4.4%) of these
seropositive subjects were also positive for
"HCV RNA" including 3 (2.6%) males and 2
(1.8%) females. Deranged liver function tests
were a feature in only 2 of these patients.
Ultrasound examination of these patients didn't
reveal any cirrhotic changes.
Journal of Pakistan Association of Dermatologists 2003; 13: 179-183.
Table 1 Age and gender wise distribution of enrolled
patients (n=114)
Age
range
(yrs)
11-20
21-30
31-40
41-50
51-60
Total
Male
n (%)
Female
n (%)
Total
n (%)
5 (4.4%)
23 (20%)
8 (7.2%)
9 (7.8%)
6 (5.2%)
51 (44.8%)
9 (7.8%)
25 (22%)
11 (9.6%)
12 (10.5%)
6 (5.2%)
63 (55.2%)
14 (12.2%)
48 (42.1%)
19 (16.6%)
21 (18.4%)
12 (10.5%)
114
Table 2 Age and sex distribution of seropositive
patients with urticaria (n = 114)
Age range
(yrs)
11-20
21-30
31-40
41-50
51-60
Total
Male
n (%)
1 (0.9%)
3 (2.6%)
1 (0.9%)
2 (1.8%)
6 (5.2%)
Female
n (%)
1 (0.9%)
5 (4.4%)
1 (0.9%)
2 (1.8%)
2 (1.8%)
9 (7.8%)
Total
n (%)
2 (1.8%)
8 (7.1%)
2 (1.8%)
2 (1.8%)
2 (1.8%)
15 (13.4%)
Discussion
Hepatitis C virus, also called "AIDS of 3rd
world", is a single stranded "RNA" virus
belonging to the family of viruses called
Togaviridae.9 HCV is a common problem
worldwide in patients receiving blood
transfusion. Injections, household contacts,
sexual transmission and occupational contacts
are the other risk factors for transmission of the
disease.10-12 The spectrum of hepatic disease
ranges from acute to chronic hepatitis, cirrhosis
and hepatocellular carcinoma. Among HCV
positive subjects, 15% recover spontaneously
and 25% have an asymptomatic disease.13
Chronic hepatitis develops in 50%,14 15-25%
patients with chronic liver disease15 and 13-51%
patients with carcinoma liver are seropositive.16
Seroprevalence of anti-HCV antibodies among
our normal population is 4-7%.9,17 Prevalence
rate among other groups stands as; blood donors
21%,18 various surgical ailments 16.5%,19
nephritic syndrome 68%,20 hemodialysis 62%,21
chronic liver disease 25%.22 Regarding
dermatoses lichen planus has a frequency of
23.5%.9 Frequency of anti-HCV antibodies in
our series was 13.4%. These figures are different
from the past studies.23,24 The frequency (13.4%)
in our study indicates a significant association
between chronic urticaria and anti-HCV
antibodies. Five (4.4%) of the subjects positive
for anti-HCV antibodies were also positive for
HCV RNA including 3 (2.6%) males and two
(1.8%) females. European trials noted much
lower rates of infection in patients with chronic
urticaria.6-8 Cribier et al.23 reported that chronic
urticaria is not significantly associated with
hepatitis C, as antibodies to HCV were found in
1 patient with chronic urticaria and in 1 of the
control group (0.9% of each group). Thus, the
frequency of anti-HCV antibodies was similar to
those of the general population. Dega et al.24
reported that among 1060 patients with hepatitis
C followed in a hepatology unit, 26 patients had
pruritus of which 5 had urticaria; one of these
had leukocytoclastic vasculitis in the setting of
cryoglobulinemia. Karlsberg et al.25 did a
systematic dermatological evaluation of 408
patients with hepatitis C: vasculitis was found in
10, only one of which had urticaria. In a
Japanese study 24% (19/79) of patients with
urticaria were seropositive.26 Thus, it can be
elucidated from these studies that the association
of urticaria and hepatitis C remains tenuous.
Whether the prevalence of HCV in chronic
urticaria in other geographic areas is similar
remains to be seen.1 In the current study,
(13.4%) patients were positive for anti-HCV
antibodies. Anti-HCV antibodies are unlikely to
be the cause of urticaria in these seropositive
patients, because of the absence of HCV RNA
and changes in liver function tests. Only (4.4%)
had HCV RNA in their blood while deranged
liver function tests were a feature in only 2
(1.8%) of these patients. Ultrasound examination
181
Chronic Urticaria: frequency of anti-HCV antibodies
of the abdomen in patients with HCV RNA
positive did not reveal any cirrhotic changes.
Cribier et al.23 claimed that hepatitis C virus is
unlikely to be the cause of urticaria in the
absence of HCV RNA and changes in liver
function tests. Therefore, in our study despite a
significant frequency of anti-HCV antibodies in
patients with chronic urticaria, HCV may not be
the underlying cause.23 As the number of
patients infected with the hepatitis C virus
(HCV) continues to increase, early diagnosis
remains paramount. Recent investigations
suggest that two cutaneous diseases, lichen
planus27-29 and porphyria cutanea tarda9 may
signal the presence of HCV infection. Chronic
urticaria may also be significantly associated
with hepatitis C virus infection.
Conclusion
There may be a significant association of
urticaria with anti-HCV antibodies. Patients with
acute or chronic urticaria of undetermined origin
be tested for HCV if features of the presentation
or epidemiology raise the level of suspicion.
Ijaz Ahmed et al.
8.
9.
10.
11.
12.
13.
14.
15.
16.
References
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Kanazawa K et al. Hepatitis C virus infection in
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Hadziyannis SJ. Skin diseases associated with
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Chuang TY, Stitle L, Brashear R, Lewis C.
Hepatitis C virus and lichen planus: a casecontrolled study of 340 patients. J Am Acad
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Chuang TY, Brashear R, Lewis C. Porphyria
cutanea tarda and hepatitis C virus: A casecontrolled study and meta-analysis of the
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Smith R, Caul EO, Burton JL. Urticaria and
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Frequency of anti-HCV antibodies in patients
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Perez Romero M, Sanchez Qugano A, Lissen E.
Transmission of hepatitis C virus (letter). Ann
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Alter MJ, Coleman PJ. Importance of
heterosexual activity in the transmission of
hepatitis B and non-A, non-B hepatitis. J Infect
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Thaler MM, C, Landers DV. Vertical
transmission of hepatitis "C" virus. JAMA 1990;
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Alter MJ, Margolis S, Krwaczynski K, Judson
FN, Mares A. The natural history of community
acquired hepatitis "C" in the United States. N
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Mattson L, Weiland O, Glaumann H. Long term
follow up of chronic posttransfusion non-A, non
B, hepatitis; clinical and histological outcome.
Liver 1988; 8: 184-8.
Malik L, Ahmed N, Butt SA et al. The role of
hepatitis "C" virus in the aetiology of
hepatocellular carcinoma in northern Pakistan- a
preliminary report. J Coll Physicians Surg Pak
1995; 5: 26-8.
Umar M, Hamama Tul Bushra, Shuaib A, Shah
NH. Spectrum of chronic liver disease due to
hepatitis "C" virus infection. J Coll Physicians
Surg Pak 2000; 10: 380-3.
Hashmi ZI, Chaudry A, Ahmad M. Healthy
volunteer blood donors; incidence of anti-HCV
antibodies. Prof Med J 1999; 6: 551-5.
Bhopal FG, Yusuf A, Taj MN, Iqbal M.
Frequency of hepatitis "B and C": surgical
patients in Rawalpindi General Hospital. Prof
Med J 1999; 6: 502-9.
Tufail HM, Shaukat A, Chaudry A et al.
Hepatitis "B and C" virus and nephritic
syndrome. Prof Med J 2000; 7: 207-12.
Shafi T, Iqbal J, Ahmad S. Prevalence of antiHCV antibodies in hemodialysis patients. Pak J
Med Res 1992; 31: 42-5.
Tariq WUZ, Hassan SZU. Prevalence of viral
hepatitis "C" in chronic liver disease in Pakistan
(abstract). The Ninth Medical Conference of
Kuwait Medical Association 1994.
Journal of Pakistan Association of Dermatologists 2003; 13: 179-183.
23. Cribier BJ, Santinelli F, Schmitti C et al.
Chronic urticaria is not significantly associated
with hepatitis C or hepatitis G infection. Arch
Dermatol 1999; 135: 1335-9.
24. Dega H, Frances C, Dupin N et al. Pruritus and
the hepatitis "C" virus. The MULTIVIRC Unit.
Ann Dermatol Venereol 1998; 125: 9-12.
25. Karlisberg PL, Lee WM, Casy DL et al.
Cutaneous vasculitis and rheumatoid factor
positivity as presenting signs of hepatitis C
virus-induced mixed cryoglobulinemia. Arch
Dermatol 1995; 131: 1119-23.
26. Kanazawa K, Yaoita H, Tsuda F, Okamoto H.
Hepatitis "C" virus infection in patients with
urticaria. J Am Acad Dermatol 1996; 35:195-8.
27. Tameez ud deen, Naqqash S, Butt AQ. Lichen
planus and hepatitis "C" virus infection: An
epidemiological study. J Pak Assoc Dermatol
2003; 13: 127-9.
28. EL Kabeer M, Scully C, Porter S et al. Liver
function in UK patients with oral lichen planus.
Clin Exp Dermatol 1993; 18: 12-6.
29. Doutre MS. Hepatitis C virus related skin
diseases. Arch Dermatol 1999; 135: 1401-3.
183
Journal of Pakistan Association of Dermatologists 2003; 13: 184-192.
Review article
Histiocytoses
Mansoor Dilnawaz, Nasser Rashid Dar
PNS SHIFA Hospital, Karachi
Abstract
Histiocytoses are an important group of dermatoses characterized by histological
infiltrates predominantly rich in cells of monocyte-macrophage origin. The present
review focuses on the salient clinical, diagnostic and therapeutic features of different
entities included in this group.
Introduction
The histiocytoses are a heterogeneous group of
diseases characterized by the accumulation of
reactive or neoplastic histiocytes in various
tissues. The majority of the signs and symptoms
of the histiocytoses may be the result of
functional activity of these cells. An abnormal or
altered regulation of histiocyte activity may be
presumed to be important in the pathogenesis of
these disorders. The present article aims to
present the diverging spectrum of these diseases
in a simplified and tabulated version.
Address for Correspondence
Surg. Lt. Commander Dr. Mansoor Dilnawaz,
D-23, Basra Lane, Lodge St, 8th Central St,
Phase II, DHA, Karachi.
E mail: drmd90@yahoo.com
184
Histiocytoses
Table 1
Histiocytoses 1-10
Tumor
Clinical aspects
Histopathology
Prognosis
Work up
Skin:
 Seborrheic dermatitis like rash on
scalp, abdomen, groins
 Yellow brown, scaly, purpuric
papulonodules
 Erosions, ulceration, scarring



Poor if
 Age > 2 years
 Widespread
For diagnosis
 Detailed history
 Thorough examination
 Skin biopsy
 Marker studies
 Electron microscopy
 Non invasive work up
Class – I Histiocytoses
Langerhans cell
histiocytosis (LCH)



Hashimoto-Pritzker variant:
Neonatal period
Resemble healing varicella
Generalized, self-limiting
Nails
 Paronychia
 Onycholysis
 Subungual expansion
 Nail plate loss
Oral
 Periodontal involvement
 Mandibular mass
Ears
 Polypoids in external ear
 Discharge, deafness
Lymphohistiocytic infiltrate
Mixed cellular infiltrate
Pautrier’s like microabscesses
Markers
 S 100 protein
 Peanut agglutinin
 Placental alkaline phosphatase
 Alpha–D mannosidase
 CD 1
Ultrastructure
 Birbeck granules
For extent
 Based on symptoms
For follow up
 Disease itself
 Opted therapies
Treatment options
Skin limited LCH:
 Topical 20% nitrogen mustard
 PUVA therapy
 Thalidomide
Multisystem LCH:
 Prednisolone 2mg/kg x 2 months
185
Journal of Pakistan Association of Dermatologists 2003; 13: 184-192.
Table 2
Histiocytoses 1-10 (Cont’d….)
Tumor
Clinical aspects
Neuroendocrine
 Cerebellar involvement
 Diabetes insipidus
 Short stature
 Hypogonadism
 Thyroid involvement
Lungs:
 Pneumothoraces
 Non specific and rare
Gastrointestinal Tract:
 Hepatomegaly
 Ascites
 Cholestatic jaundice
 Malabsorption
 Diarrhea
Bone:
 Osteolytic lesions
 Pathological fractures
 Vertebral collapse
Bone Marrow:
 Pancytopenia
 Splenomegaly
186
Histopathology
Prognosis
Work up



Chemotherapy with
vinblastine, methotrexate, 6–
mercaptopurine, etoposide,
cyclosporine
Alpha interferon
Combination
Histiocytoses
Table 3
Histiocytoses 11-13 (Cont’d….)
Tumor
Clinical Aspects
Histopathology
Prognosis
Work Up
Dermatofibroma



Firm yellow brown nodules
Dimple sign
Limbs


Storiform pattern
Factor XIIIa staining

Good


Surgical excision
Intralesional steroids
Juvenile xanthogranuloma





Onset in infancy
Asymptomatic
Sudden appearance
Spontaneous regression
Firm reddish yellow macules,
papules, plaques
Upper half of the body
Surface telangiectasia
Ulcers, atrophic scars



Touton giant cells
Lymphohistiocytic infiltrate
Mixed cellular infiltrate

Resolves in 1–5
years


As for LCH class–I
No treatment needed for skin
disease
Surgery, radiotherapy for ocular
lesions
Class – II Histiocytoses




Complications:
 Iris hemorrhages
 Secondary glaucoma
 Iritis, uveitis, proptosis
 Visceral involvement
Associations:
 Leukemias
 Urticaria pigmentosa
 Neurofibromatosis
 Neimann–Pick disease
187
Journal of Pakistan Association of Dermatologists 2003; 13: 184-192.
Table 4
Histiocytoses 14-23 (Cont’d….)
Tumor
Clinical Aspects
Histopathology
Prognosis
Work up
Benign cephalic
histiocytosis


Resemble juvenile xanthogranuloma
No mucosal or visceral involvement

Same as juvenile
xanthogranuloma

Good


As for LCH class–I
No treatment needed
Necrobiotic
xanthogranuloma


Periorbital noduloulcerative lesions
Xanthomatous plaques, ulcerations,
atrophy
Ocular involvement
Associated paraproteinemia



Touton cells
Necrobiosis
Palisading granulomas

Depends on the
extent of disease

Same as in necrobiotic
xanthogranuloma

Depends on the
extent of disease









As for LCH class–I
Treatment of paraproteinemia
Systemic steroids
Chemotherapy
Radiotherapy
Plasmapheresis
As for LCH class–I
Systemic steroids
Immunosuppressives



Xanthomatous element
Foamy macrophages
Mixed cellular infiltrate

Depends on the
extent of disease



As for LCH class–I
Treatment of paraproteinemia
Plasma exchange


Multicentric
reticulohistiocytosis
Arthropathy
 Rheumatoid arthritis like
Skin involvement
 Yellow brown papulonodules
 Extremities, face, scalp, ears
 Nail dystrophy, pruritus
Mucosal infiltration
Internal malignancies
Fatal cardiac involvement
Diffuse plane
xanthomatosis



188
Large, flat, plaque like
xanthomatous lesions
Head, neck, trunk, flexures
Associated paraproteinemia
Histiocytoses
Table 5
Histiocytoses 14-23 (Cont’d….)
Tumor
Clinical Aspects
Histopathology
Prognosis
Work up
Generalized eruptive
xanthoma



Yellow brown papules
Generalized distribution
Diabetes insipidus


Lymphohistiocytic infiltrate
Mixed cellular infiltrate

Depends on the
extent of
disease



As for LCH class–I
Treatment of symptoms
Treatment of complications
Xanthoma disseminatum





Yellow brown papules
Generalized distribution
Mucosal involvement
Systemic involvement
Diabetes insipidus


Lymphohistiocytic infiltrate
Mixed cellular infiltrate

Depends on the
extent of
disease



As for LCH class–I
Treatment of symptoms
Treatment of complications
Familial hemophagocytic
lymphohistiocytosis
(Autosomal recessive)






Fever maculopapular rash
Signs of meningeal irritation
Lymphadenopathy
Hepatosplenomegaly
Anemia
Thrombocytopenia


Lymphohistiocytic infiltrate
Lymphopenia in lymphoid
tissues

Depends on the
extent of
disease






As for LCH class–I
Splenectomy
Exchange transfusion
Chemotherapy
Marrow transplantation
Treatment of complications
Familial sea blue
histiocytosis
(Autosomal recessive)




Pigmentation
Upper half of the body
Stippled deposits on macula
Systemic involvement

Histiocytic infiltrate

Depends on the
extent of
disease



As for LCH class–I
Treatment of symptoms
Treatment of complications
Hereditary progressive
mucinous histiocytosis
(Autosomal dominant)



Red brown papules
Acral parts
Progressive


Histiocytic infiltrate
Mucinous component

Depends on the
extent of
disease



As for LCH class–I
Treatment of symptoms
Treatment of complications
189
Journal of Pakistan Association of Dermatologists 2003; 13: 184-192.
Table 6
Histiocytoses 14-23 (Cont’d….)
Tumor
Clinical Aspects
Histopathology
Prognosis
Work Up
Sinus histiocytosis with
massive lymphadenopathy
(Klebsiella, Brucella, EBV)






Massive adenopathy
Cervical adenopathy > 90%
Yellow purple papulonodules
Nasal polyps
Constitutional symptoms
Systemic involvement



Dilated sinuses
Reactive germinal centers
Lymphophagocytosis

Depends on the
extent of
disease





As for LCH class–I
Chemotherapy
X-rays
Treatment of symptoms
Treatment of complications
Virus-associated
hemophagocytic syndrome
(Herpes, adenovirus, EBV)





Sudden onset
Generalized macular rash
Constitutional symptoms
Hematological abnormalities
Other systemic involvement


Histiocytic infiltrate
Erythrophagocytosis

Depends on the
extent of
disease



As for LCH class–I
Treatment of symptoms
Treatment of complications
Malakoplakia
(Soft plaque, an
immunodeficiency disease)




Papulonodules
Abscesses, sinuses, ulcers
Mucosal involvement
Systemic involvement

Hansemann cells (histiocytes
with eosinophilic granules)
Michaelis Gutmann bodies
(histiocytes with basophilic
inclusions)

Depends on the
extent of
disease



As for LCH class–I
Treatment of symptoms
Treatment of complications
190

Histiocytoses
Table 7
Histiocytoses 24-26 (Cont’d….)
Tumor
Clinical aspects
Histopathology
Prognosis
Work up



Malignant histiocytic
infiltrate
Adnexal involvement

Poor




Constitutional symptoms
Red brown, violaceous macules,
nodules
Gum involvement
Hematological abnormalities
Extramedullary disease
Organomegaly






As for LCH class–I
Blood and marrow studies
Chemotherapy
Radiotherapy
Electron beam therapy
Marrow transplant
Malignant histiocytosis

Same as in monocytic leukemia

Same as in monocytic
leukemia

Poor

Same as in monocytic leukemia
True histiocytic lymphoma

Same as in monocytic leukemia

Same as in monocytic
leukemia

Poor

Same as in monocytic leukemia
Class – III Histiocytoses
Monocytic leukemia (M5 of
FAB classification)

191
Journal of Pakistan Association of Dermatologists 2003; 13: 184-192.
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Multicentric reticulohistiocytosis and
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associated histiocytoses: a clinical,
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Br J Dermatol 1995; 133: 71-6.
Kossard S, Winkelmann RK. Necrobiotic
xanthogranuloma with paraproteinemia. J
Am Acad Dermatol 1980; 3: 257-70.
Rosai J, Dorfmann RE. Sinus histiocytosis
with massive lymphadenopathy: a
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Analysis of 34 cases. Cancer 1972; 30:
1174-88.
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582-9.
Calverly DC, Wismer J, Rosonthal D.
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Blobstein SH, Caldwell D, Carter M.
Bone lesions in xanthoma disseminatum.
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Finan MC, Winklemann RK. Necrobiotic
xanthogranuloma with paraproteinemia. A
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Journal of Pakistan Association of Dermatologists 2003; 13: 193-201.
10th Annual Conference of Dermatology,
Bhurban 26-28 September, 2003
Pakistan Association of Dermatology held its 10th Annual Conference at Bhurban from
26 to 28 September, 2003. Abstracts of all scientific presentations during the
conference are reproduced herein.
1. Sensitivity of various laboratory
investigations in the diagnosis of Old World
cutaneous leishmaniasis
This study examines the clinical efficacy of
Nd:YAG hair removal laser at 4 and 40
milliseconds (ms) pulse durations.
Asher Ahmed Mashhood
CMH, Peshawar
Methods Ten Pakistani patients with coarse
facial hair were treated with Nd:YAG laser.
Keeping all the other parameters identical, one
side of the face was randomly treated with 4
ms pulse duration and other with 40 ms. Four
treatment sessions with 12-15 mm spot size
and fluence of 26-42 J/cm2 were performed
after 4-6 weeks. Final objective evaluation was
done 6 months after the last treatment.
Objective To find the sensitivity of Leishmanin
test, direct microscopy and leishmania culture
in the diagnosis of cutaneous leishmaniasis in
Pakistan.
Design Observational study.
Setting Skin Department, PNS Shifa and
DESTO Laboratories, Karachi.
Materials and methods Forty patients were
included in this study. Leishmanin test, direct
microscopy and leishmania culture were
performed on all these patients.
Result Leishmanin skin test was positive in all
the 40 patients (100%). Direct microscopy for
leishmania was positive in 28 patients (70%).
Leishmania culture was positive in 32 patients
(80%).
Conclusion Traditional methods of diagnosis
are very sensitive for the diagnosis of
cutaneous leishmaniasis. Leishmanin skin test
can be used as a screening test for cutaneous
leishmaniasis.
2. Comparison of 4 and 40 milliseconds
pulse durations for Nd: YAG hair removal
laser
Shahid Javaid Akhtar
Department of Dermatology, Punjab Medical
College, Faisalabad
Objectives Laser-assisted hair removal is the
therapy of choice for treatment of unwanted
hair and Nd:YAG laser system is the safest for
Asian skin. Longer pulse durations are
considered to be more effective but scientific
data evaluating this parameter alone is sparse.
Results Forty milliseconds pulse duration
resulted in an average hair reduction of 78%
compared with the baseline while 4 ms pulse
width caused an average reduction of 59%
(p<0.05).
Conclusion Longer pulse durations are more
effective in causing permanent laser hair
reduction.
3. Xeroderma pigmentosum in Larkana,
Pakistan. Clinical and histopathological
observations
Abdul Manan Bhutto, Aijaz Hussain Solangi,
Dileep Kumar.
Department of Dermatology, Chandka Medical
College, Larkana
Xeroderma Pigmentosum (XP) is a rare
autosomal recessive disorder caused by defect
in normal repair of DNA of various cutaneous
cell types damaged by exposure to ultraviolet
(UV) radiations. We present our 7-year
experience with 36 XP patients. They either
visited dermatology department, and/or were
seen during the medical camps arranged in
remote areas for patients’ welfare from 1995 to
2001. The male to female patient ratio was 1:1.
The age of patients ranged from 2 to 30 years
(mean 8.9 years). The disease (freckles and/or
photophobia) started at the age of 6 to 24
months. All the patients had mild to severe
freckles on the exposed parts of the body and
193
10th Annual Conference of Dermatology: abstracts
the majority were sensitive to sunlight.
Seventeen patients had changes like actinic
keratosis, keratoacanthoma, fissures and
ulcerative nodules on the exposed parts of the
body. Four patients had large ulcers along with
mass formation and severe pigmentation on the
face, neck and head. Twenty-nine patients
developed ocular symptoms like photophobia,
conjunctivitis, corneal keratitis and lid ulcer.
One patient was seen with complete loss of
vision. Histopathological findings revealed that
6 patients had squamous cell carcinoma (SCC)
on different parts of the body like face, head,
ear and lip. Two to four siblings were affected
in four families. No neurological abnormalities
were observed in our patients. All the patients
were treated symptomatically. Two patients
died due to extensive SCC.
4. Neurofibromatosis and Caroli's disease an extremely rare combination
Arfan ul Bari, Simeen ber Rahman‫٭‬
PAF Hospital, Sargodha
‫٭‬Military Hospital, Rawalpindi
Background Neurofibromatosis (NF) has
fascinated physicians and scholars for many
centuries. This condition is one of the common
autosomal dominant disorders in human beings
that affects the bone, the nervous system, soft
tissue, and the skin. NFl accounts for 96% to
97% of all cases of NF and results from defects
in the NFl gene on chromosome 17. NFl is
usually diagnosed by cardinal dermatologic
features but it can be compounded by a broad
spectrum of complications and associations.
We present a florid case of NF who presented
with unusual symptoms and was found to have
associated Caroli's disease, which is a rare
congenital disorder of the intrahepatic bile
ducts.
Case history A 43-year-old female presented
with generalized pruritus for last six months
and moderate to high-grade fever for one
week. On physical examination she was found
to have multiple neurofibromas, café-au-lait
macules, axillary freckles and Lisch nodules.
On abdominal ultrasonography multiple
dilatations and stasis was seen in hepatobiliary
tree and a diagnosis of associated Caroli's
disease was made. She was initially managed
conservatively and then referred to surgical
specialist for further evaluation and
management.
Conclusion Caroli's disease is a rare congenital
disorder and its association with NF is even
rarer. To date only one such case has been
reported in literature.
194
5. Etiologic break-up in cases of
erythroderma in our community as assessed
by clinical and histopathological evaluation
Wajiha Irshad, Afaq Ahmed, Maqsood
Anwar
Department of Dermatology, PIMS, Islamabad
Background Erythroderma is a fairly common
entity presenting to dermatology outpatients,
sometimes also landing in emergency rooms.
Few original patient series have been
published
locally
and
internationally.
Moreover, the relative frequency of different
etiologies is expected to change over the years.
Objectives 1. To study the etiologic break up
of erythroderma in our community, aided by
clinical evaluation and histopathological
examination. 2. To assess the frequency of
cases remaining undiagnosed despite thorough
clinical and histopathological evaluation.
Methods 50 consecutive adult (38 male, 12
female) patients with erythroderma presenting
to the dermatology outpatients from November
2001 to December 2002 were enrolled into this
prospective study after obtaining verbal
consent. Personal and clinical data were
entered into a pro forma. Skin biopsy was
taken and hematoxylin and eosin stained
sections were analyzed in the hospital's
laboratory. Final diagnosis was reached by corelating clinical and histopathological findings.
Results The final break-up of causes showed
that eczemas of different types were found in
17(34%) patients, psoriasis was responsible in
14(28%) and drugs in 4(8%) of patients.
Pemphigus foliaceous was found in 2(4%)
patients whereas cutaneous T-cell lymphoma,
secondary skin infiltration by non-Hodgkin's
lymphoma, pityriasis rubra pilaris and
ichthyosis were responsible in 1(2%) patient
each. 9(18%) patients remained undiagnosed
despite thorough clinical and histopathological
evaluation of H&E stained sections.
Conclusion
Pre-existing
dermatoses
(predominantly eczema and psoriasis and less
commonly pemphigus foliaceous, pityriasis
rubra pilaris and ichthyosis) constituted the
largest group of erythrodermic patients
followed by the idiopathic group. Drugs
accounted for the rest, whereas disorders
related to malignancy were present in a
minority.
Journal of Pakistan Association of Dermatologists 2003; 13: 193-201.
6. Zosteriform lichen planus: a new variant
of a common disorder
Arfan ul Bari, Simeen ber Rahman*
PAF Hospital, Sargodha
* Department of Dermatology, Military
Hospital, Rawalpindi
Background Ordinarily, Lichen planus (LP) is
normally easily recognizable but sometimes it
may be disguised. It has been reported to occur
in the scars of previous herpes zoster lesions.
Zosteriform pattern in LP, without evidence of
herpes zoster, is an extremely rare occurrence.
Materials and methods Six patients of varied
ages presented with grouped lichenoid lesions
on various regions of the body with no
previous or concomitant history of herpes
zoster on the involved site or elsewhere on the
body. Lesions were clinically assessed and
laboratory investigations including blood CP,
blood sugar, serum liver function tests,
serology for hepatitis B & C, serum urea and
creatinine were carried out. Skin biopsies were
also performed for histopathological studies in
all cases.
Results All patients were young to middleaged males. No associated systemic illness was
seen in any patient. Skin lesions were
suggestive of LP. Laboratory investigations
were within normal limits in all patients except
one, who was positive for hepatitis C. Skin
biopsies revealed classical changes of lichen
planus in all cases.
Conclusion Linear lesions following lines of
Blaschko have not been so uncommon, but
zonal or zosteriform distribution of LP lesions
without koebnerization is a very rare
occurrence. Exact etiology of this unusual
pattern could not be ascertained. The possible
cause could be an unknown drug, food or a
form of blashkitis. Zosteriform LP is an
emerging new variant, which should be looked
for in clinical practice.
7. Beaded papular epidermal hyperplasia of
neck
Khalid Mehmood
Military Hospital, Rawalpindi
Case summary: A 14-year-old school girl
presented with one-year history of gradually
progressive, asymptomatic, linearly arranged,
fancifully symmetrical, skin colored multiple
beaded papules around the front and sides of
her neck. There were no lesions elsewhere on
her body. She did not have family history of
similar lesions. A skin biopsy revealed broad
papillomatosis, mild acanthosis and a few
vacuolated cells but no hypergranulosis or
verrucae
bodies.
The
dermis
was
unremarkable. The lack of family history and
absence of classical histological features ruled
out a diagnosis of epidermal dysplasia
verruciformis. There was no sebaceous gland
hyperplasia in the dermis as seen in juxtaclavicular beaded papules of neck. The elastic
tissue stains did not reveal degenerated elastic
fibres in the dermis as seen in pseudoxanthoma
elasticum and pseudoxanthoma-like papillary
dermal elastolysis of the neck. The
morphology and distribution of the lesions was
unlike that of dermatosis papulosa nigra. A
miniature form of similar lesions was observed
in another eleven young girls and one young
man. This most likely represents a normal
physiological variation. Since this entity does
not fit in any of the known dermatosis,
therefore a descriptive name "Beaded Papular
Epidermal Hyperplasia of Neck" has been
suggested.
8.
Retinitis
pigmentosa-like
oral
pigmentation in association with retinitis
pigmentosa
Khalid Mahmood
Military Hospital, Rawalpindi
Case summary Four cases of pigmentation of
oral mucosa associated with retinitis
pigmentosa belonging to the same family are
being reported. The father and three out of six
siblings were affected. The pattern and colour
of pigmentation in the buccal mucosa was very
similar in appearance to that of retina in
retinitis pigmentosa. The pattern of
pigmentation in the oral mucosa in the first
instance prompted the author to ask for history
of night blindness and led to the examination
of fundus with resultant diagnosis of retinitis
pigmentosa in four family members who were
previously unaware of its presence. The three
siblings who did not have retinitis pigmentosa
had no pigmentation in the oral mucosa. This
suggests an association of retinitis pigmentosalike oral pigmentation with retinitis
pigmentosa. Although there are many
dermatological associations of retinitis
pigmentosa but to the best of my knowledge
no such association, as reported here, has been
previously described.
195
10th Annual Conference of Dermatology: abstracts
9. Non Hodgkin’s lymphoma
Umair M Bajwa, Naveed Ahmad
Social Security Hospital, Lahore
Lymphomas are designated as primary or
secondary on the basis of their origin in the
skin or extra-cutaneous tissues. Cutaneous
lymphomas are classified according to their
cell type of origin. Case of a 55-year-old male
is described who had big masses on his
forehead, side of face and axillae for the last
one year. Biopsy was taken from all the three
sites for histopathology. The diagnosis of nonHodgkin’s follicular centrocytic lymphoma
was made. Patient was put on chemotherapy.
He
received
regular
pulses
of
cyclophosphamide, doxorubicin, vincristine,
oncovin and prednisolone. His response was
remarkable and only after three pulses the
lesions decreased by 75%. This type of
lymphoma carries good prognosis and
prolonged remission is usually achieved. Five
years survival is around 60%.
10. Contact reactions in hospital workers
Tanzeela Khalid, Arif Maan, Syed
Muhammad Azam Bokhari
Department of Dermatology, Punjab Medical
College, Faisalabad
Background Contact dermatitis accounts for
more than 90% of all occupational skin
diseases. Hospital workers are exposed to a
wide variety of allergens and irritants as a part
of their occupational environment.
Objectives Aim of the study was to determine
the pattern of contact dermatitis in hospital
workers in our setup.
Study Design 732 hospital workers, including
doctors and paramedical staff were screened
using a questionnaire. All those with the
clinical suspicion of allergic contact dermatitis
were patch tested. Results were read at 48,72
and 120 hours and were interpreted according
to International Contact Dermatitis Research
Group criteria and relevance correlated.
Results Out of the 732 hospital workers that
were screened 219(30%) reported contact
reactions (including allergic contact dermatitis,
irritant contact dermatitis and contact
urticaria). 109(50%) had irritant contact
dermatitis whereas 105(48%) were diagnosed
as having allergic contact dermatitis. Five (2%)
had contact urticaria. Out of those patch tested,
nickel sulphate was the most frequent allergen.
196
Conclusion Irritant contact dermatitis is more
common than allergic contact dermatitis
among hospital workers in our setup.
11. Effect of contact dermatitis
dermatology life quality index
on
Zahida Rani, Ijaz Hussain, Tahir Saeed
Haroon
Department of Dermatology, King Edward
Medical College/Mayo Hospital, Lahore
Background Dermatology Life Quality Index
(DLQI) is a self-administered general quality
of life instrument, which has been widely used,
in different dermatological disorders including
contact dermatitis, which is a chronic recurrent
dermatosis with physical and psychosocial
handicap.
Objective To find out the impact of contact
dermatitis on quality of life in patients by
using DLQI and to correlate with various
factors e.g. duration, extent of disease etc.
Patients and methods DLQI questionnaire was
administered to 87 (60 females and 27 males)
consecutive patients with contact dermatitis
who were seen at the Patch Test Clinic,
Department of Dermatology, Mayo Hospital,
Lahore, from January to June, 2000. The
higher the score of DLQI, the poorer was the
quality of life.
Results The mean DLQI score was 11.29±6.8.
The mean score was 9.48±5.38 in males and
12.1±7.3 in females. The mean scores were
higher in young (21-40 years), married,
females with shorter duration of disease.
Conclusion Contact dermatitis has more
devastating effect on young and married
females.
12. Are young medical graduates equipped
with required minimum dermatology
knowledge?
Muhammad Jahangir
Dermatology Department,
Medical College, Lahore
Allama
Iqbal
Objective The purpose of this study was to
assess the level of knowledge of our medical
graduates about the common dermatoses
prevalent in this community.
Subjects and methods Medical graduates from
various medical colleges, based in Lahore and
doing their first year of house job were
enrolled in this study. Their knowledge of
common dermatoses was assessed by a written
Journal of Pakistan Association of Dermatologists 2003; 13: 193-201.
test. The question-paper consisted of a
combination of multiple choice, single best and
matching type of questions. A panel of three
consultant
dermatologists
involved
in
undergraduate
teaching
devised
every
component of the question-paper.
Results One hundred and ninety eight subjects
were enrolled in this study. They were working
as house officers in various departments of
Jinnah, Sir Ganga Ram, Services, Mayo and
Lahore General Hospitals in Lahore. Analysis
of data revealed that 34% had never entered a
dermatology unit for training during their
graduation. Only 35% of the subjects had
attended up to three-quarters of dermatology
lectures and clinical sessions. Only 26% of
medical graduates achieved the results
indicative of desired minimum knowledge of
dermatology. The remaing 74% were grossly
deficient in their dermatological awareness.
Conclusion The majority of our young medical
graduates lack the minimum dermatological
knowledge.
13. Chronic mucocutaneous candidiasis - a
fatal disease
Rubina Qureshi, Faisal Malik, Zafar Ullah
Kundi
PIMS, Islamabad
Background
Chronic
mucocutaneous
candidiasis, infection of mouth, skin and nail
refractory to conventional therapy is a rare
disorder. It has many varieties. Some of them
are subtle and easily missed problems. Yet
their diagnosis is vital for the life of the
patient.
Brief History The patient presented to
Dermatology OPD for the failure of
conventional treatment for candida infection of
airways. This 11-months-old female child had
presented to a pediatrician with hoarseness of
voice since 1 month of age, deformed nails,
scaly skin lesions and ulcers on ear and back of
neck since 5 months. Her elder sister died at 6
months of age with hoarseness of voice but
without skin lesions, and a brother died at two
and a half years of age with similar condition.
She was treated with topical antifungal and
griseofulvin in the ward. Tracheostomy was
done when she went into acute respiratory
distress in the hospital. Histopathology of the
tracheostomy specimen showed mycelial
growth and spores of candida. She was
investigated for immunodeficiency and
endocrinopathies, which were normal. She
responded well to itraconazole and was
discharged from the hospital. Her hoarseness
improved after 4 weeks of treatment.
14.
Deep
mycosis
following
immunosuppression for pemphigus vulgaris
Simeen Ber Rahman, Javeria Hafeez
Dermatology Department, Military Hospital
Rawalpindi
We report a case of pemphigus vulgaris and
NIDDM, well controlled on drugs, who
developed large nodular swelling over the
chest. A large cheesy mass was expelled from
the swelling, which histopathologically
revealed numerous branching septate fungal
hyphae. Aspergillus flavus was isolated on
Sabouraud dextrose agar. Chest radiograph
revealed chronic inflammatory changes in lung
fields. His bronchial washings were positive
for AFB. The patient has been placed on
antifungal and antituberculous treatment to
which he has responded well.
15. Effect of LPIR alexandrite laser on
Asian skin & safety of laser in pregnancy
Haneef Saeed
Skin Care and Laser Clinic, Karachi
In 800 women clients with the clinical
diagnosis of hirsutism, 18 (eighteen) were
pregnant when first epilation was started and
23 conceived during the laser epilation
sessions. Age ranged from 20 to 40 years and
the clients were from low socioeconomic to
upper middle class. Most of them were
housewives. Hormonal status was normal
during pregnancy. Two of them were suffering
from gestational diabetes mellitus. Half the
ladies had Hb% less than 10 gm%. Period of
study was from Ist October, 2000 to 30th
April, 2002. Antenatal, natal and postnatal care
was carried out in different hospitals and
maternity centers. After delivery the neonates
were thoroughly examined by a paediatrician;
their apgar scores ranged from 6/10 to 8/10.
All were normal and there was no
complication during antenatal, natal or
postnatal period. No congenital abnormality
was detected.
Treatment plan Treatment was done with LPIR
alexandrite laser and skin type was III to V
associated with acne in 10% cases and
melasma in 14% of cases. Treated areas: chin
36%, upper lip 28%, sideburns 20%, eyebrows
10%, arms 2%, armpits 2%, forehead 2%. 6 10 treatments at 4 - 8 week intervals were
carried out. Hair growth and density reduced
up to 80% but a remarkable increase in the
197
10th Annual Conference of Dermatology: abstracts
length of hair was noted after 6 treatments.
Mild burns were noted in 2% clients.
Hyperpigmentation and postinflammatory
hypopigmentation were observed specially in
skin type V.
Conclusion Laser epilation with LPIR
alexandrite laser is safe in pregnancy and is
effective by 70% to 80% in Asian skin.
16. FNAC in granulomatous and malignant
skin conditions
Eanas Bader, Afaq Ahmad
Dermatology Department, PIMS, Islamabad
Background Granulomatous and malignant
skin diseases can be diagnosed by fine needle
aspiration cytology (FNAC) and skin biopsy.
FNAC is a quick, comfortable, accurate and
reliable method of sampling with minimal
complications and patient discomfort. When an
adequate specimen is obtained, a definitive
diagnosis can be established.
Objective To study the yield of FNAC in
granulomatous and malignant skin disorders.
Methods Twenty patients with the clinical
diagnosis of granulomatous or malignant
disease irrespective of age and gender were
studied. FNAC was performed keeping the
needle horizontal or slightly oblique. Slides
were stained with hematoxylin and eosin and
studied.
Results Out of 20 FNAC 80% were positive. In
20% of cases the result was hemorrhagic
aspirate.
Conclusion FNAC is a diagnostic method that
is of great value in the rapid, reliable and costeffective diagnosis of granulomatous and
carcinomatous lesions.
17.
Secondary
syphilis
mimicking
palmoplantar pustular psoriasis: an unusual
clinical presentation
Attiya Rahman, Zafar Iqbal Sheikh, Simeen
Ber Rahman
Department
of
Dermatology,
Military
Hospital, Rawalpindi
Pustular lesions in the secondary stage of
syphilis are rare. We report a 38 years old
Pakistani male with secondary syphilis who
had pustular and erythematous scaling lesions
on the palms and soles which closely
resembled palmoplantar pustular psoriasis. The
198
patient's serology and histopathology for
syphilis was positive and the lesions cleared
with injection benzyl penicillin.
18. Topical calcipotriol vs. oral psoralenUVA (PUVA) and topical calcipotriol in the
treatment of vitiligo in type-IV skin
Atif Shehzad, Khawar Khurshid, Sabrina
Suhail Pal, T.S. Haroon
Department of Dermatology, King Edward
Medical College/Mayo Hospital, Lahore
Background The treatment of vitiligo is a
challenging task for dermatologists. Although
several therapeutic modalities have been tried,
till date there is no convincing treatment of the
disease. Psoralen-UVA (PUVA) has an
established role in the treatment of vitiligo.
Topical calcipotriol alone and in conjunction
with PUVA has shown good results in recent
international studies in the treatment of type-I
and II vitiliginous skin.
Objective To assess the role of topical
calcipotriol alone and in conjunction with
PUVA in the treatment of vitiligo in type IV
skin.
Patients and methods This study was
conducted in the Department of Dermatology,
Mayo Hospital, Lahore, from 1st June to 1st
December 2002. Sixty patients of vitiligo (26
males and 34 females), aged 12-60 years and
involving <30% of body surface area were
enrolled. All the patients were randomly
divided into two equal groups. Group-I
patients were treated with twice daily
application of topical calcipotriol for six
months, whereas in group-II patients in
addition to topical calcipotriol, photochemotherapy was advised thrice a week for
the same duration. Response was graded as
follows: G0-no response (0%), G1-poor
response (1-25%), G2-fair response (26-50%),
G3-good response (51-75%), G4-excellent
response (76-100%).
Results Seventy per cent of the patients in
group II showed excellent response, whereas
none of the patients in group I had similar
response.
Conclusion We conclude that topical
calcipotriol alone has no role in the treatment
of vitiligo in type-IV skin but when combined
with PUVA may potentiate its efficacy,
leading to an excellent response in >2/3 of the
patients.
Journal of Pakistan Association of Dermatologists 2003; 13: 193-201.
19. Frequency and complications
irrational use of topical steroids
dermatophytosis
of
in
Maqsood Anwar, Nosheen Rizwan, Nabeel
Riaz Sheikh*
Department of Dermatology, PIMS, Islamabad
* King Edward Medical College, Lahore
Background Dermatophytosis or ringworm
infections are very common skin infections,
frequently maltreated. A study was conducted
in department of dermatology PIMS to
determine the frequency of irrational use of
topical steroids by the doctors and patients.
Objectives 1. To determine frequency of
topical steroids used in dermatophytosis. 2. To
find out frequency of irrational use by doctors
and indiscriminate use by patients. 3. To
observe complications caused by topical
steroids. 4. To know the type of steroids used.
Methods Over a period of six months 154
diagnosed cases of dermatophytosis were
included in the study. Diagnosis was based on
clinical assessment and KOH preparation. Data
was collected by a questionnaire, analyzed and
presented by frequency and percentage.
Results Total 154 cases from the 14 districts of
Pakistan and AJK were enrolled. Male:female
ratio was almost 1:3. 88 (57.1%) patients had
applied steroid before coming to the OPD. 54
(35.1%) had applied betamethasone, 21
(13.6%)
clobetasol,
8
(5.2%)
1%
hydrocortisone and 4 various other forms of
fluorinated steroids. 52 (33.8%) applied steroid
following a doctor's prescription, 36 (23.4%)
applied on their own. 43 (27.9%) out of 88
presented with plaques of tinea incognito, and
21 (13.6%) presented with secondary bacterial
infections.
Conclusion topical steroids are commonly
prescribed by doctors or used on their own by
patients. This irrational use not only aggravates
and complicates the disease, which in turn
leads to prolonged treatment time but also
increases the cost of treatment.
20. Patients’ perceptions about acne. A
questionnaire study in 100 patients with
acne.
knowledge about the disease leads
maltreatment, enhancing morbidity.
to
Objective The objective of the study was to
find out and document patients' perceptions
about various aspects of acne.
Patients and methods A questionnaire survey
was conducted among one hundred
consecutive patients suffering from acne
attending skin OPD, Jinnah hospital, Lahore.
The questionnaire consisted of 22 items about
different aspects of acne. The data was
tabulated and analyzed with the help of a
database.
Results Misperceptions about acne were
common in study population. Food (81%) and
indigestion (42%) were considered to
aggravate acne. Relieving factors included;
repeated washing of face (42%), topical
steroids (32%), beauty creams (25%), marriage
(19%) and emollients (15%). 75% thought
genetic/familial background unimportant in
causation of acne. Acne was conceived
contagious by 42%.
Conclusion Misconceptions are common n
patents with acne. There is a need to
incorporate proper instructional strategies as a
part of acne treatment.
21. Bowen's disease of male breast - A rare
entity.
M.A. Zahid
Department of Surgery, PIMS, Islamabad
A case of Bowen's disease of the male breast is
presented. Bowen's disease, although common
in other parts of the body, has not been
reported on the male breast disease in the
surgical literature. Bowen's disease is a slowly
enlarging erythematous patch with sharp but
irregular outline; within the patch are general
areas of scaling and crusting referred as
intraepidermal squamous cell carcinoma, or
squamous cell carcinoma in situ. There is a
full-thickness anaplasia of the epidermis, with
loss of the normal maturation of its
components.
22. Irritation with toilet soaps
Iqbal
Muhammad Arif Maan
Department of Dermatology, Punjab Medical
College / DHQ Hospital, Faisalabad
Background Acne is one of the commonest
dermatoses. Misperceptions and inadequate
Irritation with soaps and detergents is well
documented
and
many
cosmaceutical
companies claim safer soaps. We planned this
Tariq Rashid
Dermatology Department,
Medical College, Lahore
Allama
199
10th Annual Conference of Dermatology: abstracts
study to evaluate irritation from different
soaps.
Objectives To evaluate justification for
prescription or advice about soaps by
dermatologists.
Materials and methods This observational
study was carried out at the Department of
Dermatology, Punjab Medical College,
Faisalabad from 1st January 2002 to 30th June
2002. Two hundred subjects were evaluated
with the help of a questionnaire. One hundred
patients selected randomly from the patients
attending OPD and one hundred healthy
volunteers
selected
from
various
undergraduate colleges were enrolled. All
subjects who could understand the study and
give informed consent were included. The
findings were analyzed with computer using
Microsoft excel.
Results Out of 200 subjects under study there
were 150 females and 50 males. 44% females
and 38% males developed irritation with soaps.
Maximum number of subjects reported
irritation from Lux (38%) and Safeguard
(18%). 76% females and 90% males preferred
to consult a dermatologist about the use of
soap.
23.
Histological
spectrum
and
clinicopathological correlation of cutaneous
granulomas: our experience at Mayo
Hospital, Lahore, Pakistan.
Faria Asad, Sabrina Suhail Pal, Tahir Saeed
Haroon
KEMC/Mayo Hospital, Lahore Pakistan
Background Granulomatous disorders are
commonly encountered in a tertiary care
hospital. Diagnosis is rather difficult and
unreliable purely on clinical grounds. Though
the microscopic findings of different
granulomas have some similarities, but
histopathology is still the most important
laboratory investigation required to confirm
the diagnosis. Differentiating infective from
noninfective granulomas has an impact on
treatment and prognosis.
Objectives To study the histological types and
clinicopathological correlation of cutaneous
granulomas.
Materials and methods It was a retrospective
study carried out in the Department of
Dermatology, Mayo Hospital, Lahore, from
August, 2001 to June, 2003. Skin biopsies of
71 clinically diagnosed cases were subjected to
200
histopathological examination, special stains
(Ziehl-Neelsen, periodic acid Schiff, Gram and
Giemsa), serological study and fungal culture,
as and when required.
Results Out of 71 cases, the different varieties
of cutaneous granulomas, were as follows. The
most common granuloma proved on
histopathology was tuberculosis (53.5%),
followed by leishmaniasis (12.7%). Sarcoid
and fungal granulomas were seen in 9.9%
cases each. In 7% specimens, the
histopathological features were those of
leprosy. Necrobiotic granulomas were found in
4.2% cases while there were 2.8% cases of
granulomatous cheilitis.
Conclusion
The
commonest
clinical
presentations
of
granulomas
were
erythematous plaques and in more than half the
cases
histopathological
diagnosis
was
tuberculosis.
24. Treatment of depressed scar marks
without surgery
Robina Qureshi
Dermatology Department, PIMS, Islamabad
Background The treatment of depressed icepick scar marks especially on face has been a
challenge to the clinicians for ages. Recently
cosmetic surgery has brought new horizons in
the form of dermabrasion, excision of scars
and collagen injections. All are expensive
treatments with improvement ranging from 3075%.
Objective The objective of the study was to
design an inexpensive strategy of a new
concept of treatment for scars due to acne,
chicken pox, and trauma.
Basic
concept
&
methodology
Reepitheliazation & collagen formation followed
by irritation and activating old scars. Reepitheliazation and neocollagen formation is
achieved with daily topical and/or injectable
solcoceryl and activation of the old scars is
done with bi-weekly superficial and/or mid
dermis chemical peels. Results are recorded
and photographed.
Conclusion The procedure is fairly effective. It
is an out patient office procedure, painless and
cost effective. Chances of side effects are
minimal and post inflammatory hyper
pigmentation is not noted in any patient.
Improvement ranges from 50-75% within three
months of treatment of old scars whereas in
Journal of Pakistan Association of Dermatologists 2003; 13: 193-201.
new scars it is even better in a shorter period of
time.
25. Efficacy of itraconazole in the
management of dermatological fungal
infections
Eanas Bader, Riaz Sheikh
Dermatology Department, PIMS, Islamabad
Background Itraconazole is an old antifungal
drug. We wanted to know its efficacy in our
regional population presenting in PIMS.
Objective The objective of this study is to
study the efficacy of itraconazole in
dermatological
fungal
infections
and
onychomycosis.
Methods Twenty-five patients with different
skin and nail fungal infections were studied
according to specific inclusion and exclusion
criteria. All patients with skin involvement
(tinea corporis, tinea pedis, tinea cruris and
pityriasis versicolor) were given 100 mg of
itraconazole twice a day for one week. Patients
with onychomycosis were given 2 pulses with
200 mg twice a day for 7 days (one pulse per
month). The patients were reviewed every 3
weeks. Results were assessed on the basis of
improvement in signs and symptoms and
disappearance of the lesions. Side effects
experienced by the patient were also enquired
and noted in a pro forma.
Results Out of 25 cases 8 were of
onychomycosis, 5 with tinea corporis, and 7
with pityriasis versicolor, 4 with tinea pedis.
Out of 8 cases of onychomycosis 62% started
showing improvement but 37% did not show
any improvement after 2 pulses. In rest of 17
cases 29% showed mild improvement while
70% showed marked improvement.
Conclusion Itraconazole is an effective
antifungal drug. Its efficacy is better in tinea
corporis and tinea cruris than in tinea pedis.
201
Journal of Pakistan Association of Dermatologists 2003; 13: 202-203.
Surgical Pearl
Useful electrode modification for
electrosurgery
Ahsan Hameed
Department of Dermatology, Shifa College of Medicine , Islamabad
Electrosurgery is increasingly being used
in dermatology for the treatment of
various skin disorders like, warts, milia,
molluscum contagiosum, skin tags and
electroepilation etc. In Pakistan it becomes
costly to use disposable electrodes for
every
patient,
especially
for
electroepilation, which results in patient
anxiety and concern about transmissible
diseases like hepatitis and AIDS.
A convenient and cheap modification to an
existing standard electrode can be made as
follows: A needle threader as shown in
Figure 1a is used for the purpose. It is cut
with normal scissors along the line and the
distal end wrapped around the exposed
end of any standard electrode (Figure 1c).
The attachment is secured with a narrow
strip of a normal adhesive surgical tape
wound tightly so as to maintain a good
electrical connection. The fine steel wire
of the needle threader is cut with scissors
so as to achieve the desired length (Figure
1d). The fine needle electrode is now
ready for use, for electroepilation or
electrodessication.
It is important to cut the fine steel wire at
an angle so as to leave the cut edge
beveled. This makes penetration of the
Address for Correspondence
Dr. Ahsan Hameed,
Associate Professor of Dermatology,
Shifa College of Medicine, H-8/4, Islamabad.
E mail: ahsanhameed23@hotmail.com
202
needle easy. The preparation takes only a
few minutes, and therefore, a new needle
can be used for every patient, thus making
the system disposable at a very low cost. If
electrodes cannot be spared for this
purpose, or if the clinician is unusually
busy, then steel nails can be used instead.
Steel nails are available in different sizes,
the appropriate size that fits the hand piece
of the machine can be selected. The flat
head of the nail is cut with pliers to
achieve the desired length, and the needle
threader wrapped as described earlier. A
number of such electrodes can be prepared
in advance and used as and when required.
The cost of the needle threader is Rs. 5
only, and is easily available in most
general
stores.
Like
conventional
electrodes, the fine wire of the threader is
made of stainless steel which does not rust
and does not cause any adverse effects like
tattooing etc.
Such electrodes come in handy for
electroepilation or electrodesiccation of
small
lesions
like,
molluscum
contagiosum, spider nevi, milia, tiny warts
and comedones etc., where the needle can
actually be inserted into the lesion and the
lesion burnt from within which gives
better results.
Useful electrode modification for electrosurgery
Ahsan Hameed
Figure 1 Steps in the modification of an electrode, a) a needle threader which has to be cut along the
line as shown, b) cut distal end of the needle threader, c) a sample of a standard electrode which can be
modified, and d) needle threader cut to proper dimensions and wrapped around the electrode, ready for
use.
Pakistan Association of Dermatologists is holding its Silver Jubilee Conference at
Karachi from 9th to 12th December, 2004 at Karachi. JPAD will publish a special
issue on this historic occasion. Readers are requested to fully contribute about
the achievements/challenges to dermatology in Pakistan, and history of and
achievements by their departments. Manuscripts should reach the Editorial
Office by 30th June, 2004.
203
Journal of Pakistan Association of Dermatologists 2003; 13: 204-207.
Case report
Neurofibromatosis type 1 with generalized
pruritus
Arfan ul Bari, Humayun Agha,* Simeen ber Rahman**
PAF Hospital, Sargodha.
* Combined Military Hospital, Peshawar
** Dermatology Department, Military Hospital, Rawalpindi
Abstract Neurofibromatosis is a neurocutaneous condition that can involve almost any organ
system. Presenting signs and symptoms may vary widely. We describe a patient who
presented with generalized pruritus that was not responding to conventional
antihistamines or topical steroids, but responded very well to ketotifen. A brief review
of the disorder is also made.
Key word
Neurofibromatosis, pruritus.
Introduction
Neurofibromatosis is an autosomal
dominant disorder that affects the bone,
the nervous system, soft tissue, and the
skin. At least 8 different clinical
phenotypes of neurofibromatosis have
been identified and are linked to at least 2
genetic disorders. Clinical manifestations
increase over time. Two major subtypes
exist: neurofibromatosis 1 (NF-1), which
is the most common subtype and is
referred to as peripheral NF, and
neurofibromatosis 2 (NF-2), which is
referred to as central NF. These
descriptions are not especially accurate
because NF-1 often has central features.1-3
NF-1 is the most common type, affecting
about 90% of those people with NF. It was
first described by a German doctor named
Frederich von Recklinghausen in 1882 and
thus the condition is also known as von
Recklinghausen's disease.4 Worldwide,
NF-1 occurs in approximately 1 of 2500Address for Correspondence
Squadron Leader Dr. Arfan ul Bari,
Consultant Dermatologist,
PAF Hospital, Sargodha.
E mail: albariul@yahoo.com
204
3300 live births, regardless of race, sex, or
ethnic background. The carrier incidence
at birth is 0.0004, and the gene frequency
is 0.0002. Male to female ratio is equal.
This disease can involve various body
systems over time. Signs can range from
benign cutaneous manifestations to
profound disfigurement.1-3 People who are
affected by NF1 have a mutation in a gene,
called NF-1 gene, which is on
chromosome 17.5 A number of different
mutations have been found in people
affected with NF1. In about half of all
cases, NF is inherited from an affected
parent. The remainder results from
spontaneous mutation and the affected
person is then the first person in a family
to be affected with NF. That person will
then be able to pass on the mutation to
his/her children.6,7 The mortality rate is
higher than that of the healthy population
because of the increased potential for
malignant transformation of diseased
tissues and the development of
neurofibrosarcoma. Patients with NF-1
have an estimated 3-15% additional risk of
malignant disease in their lifetime.8,9 The
most common characteristic of NF-1 is the
presence of flat, brown patches on the skin
Neurofibromatosis type 1 with generalized pruritus
(called "cafe-au-lait" spots which means
"coffee with milk" in French). These
usually arise in childhood. People with
NF1 also develop freckling under the arms
and in the groin. These spots and freckles
pose no threat to a person's health. The
neurofibromas which are characteristic of
this disorder may grow on nerves in many
different parts of the body: they may occur
on, or just under, the skin, and
occasionally in deeper parts of the body.
Neurofibromas can appear at any age but
very often appear during adolescence and
in women during pregnancy. They are
usually not painful. Overgrowths of
groups of nerves are called plexiform
neurofibromas. These benign (noncancerous) tumours are often located in
the deeper tissues and occur in around
25% of people with NF-1 and may cause
cosmetic disfigurement. Small clumps of
pigmented cells in the iris of the eye
(called Lisch nodules) are often seen.
However, these nodules can only be
detected by an ophthalmologist using a slit
lamp. They do not affect vision. Bony
changes, particularly in the long bones of
the lower legs, can produce bowing in the
long bone and/or fractures known as a
pseudoarthrosis or artificial joint. This is a
rare complication of NF1, affecting about
2% of patients and may require surgical
correction. The majority of children with
NF1 will have a degree of learning
difficulty, particularly in the area of
language and reading ability. The
diagnostic criteria are met if 2 or more of
the features listed are present: (i) Six or
more café au lait macules larger than 5
mm in greatest diameter in prepubertal
individuals and those larger than 15 mm in
greatest
diameter
in
postpubertal
individuals,
(ii)
Two
or
more
neurofibromas of any type or 1 plexiform
A.U. Bari et al.
neurofibroma, (iii) Freckling in the
axillary or inguinal regions, (iv) Optic
glioma, (v) Two or more Lisch nodules
(iris hamartomas), (vi) A distinctive
osseous lesion, such as sphenoid dysplasia
or thinning of the long bone cortex, with
or without pseudoarthrosis, (vii) A firstdegree relative with NF-1 according to the
above criteria.1-4,8,10 Treatment of NF1 is
primarily symptomatic and cure is not yet
possible. Multicenter trials of medications
to limit growth of optic nerve gliomas and
plexiform neurofibromas are underway.3,10
Case history
A 57-year-old lady reported in skin outdoor of PAF Hospital, Sargodha with
history of generalized itching all over the
body but more marked over her trunk.
Itching started about two years ago but
increased in intensity during last six
months. It was episodic, would occur at
any time and would not subside
satisfactorily with various antihistamines
and topical steroidal preparations. There
was no history of jaundice, weight loss,
urinary or bowel complaints. On physical
examination she was found to have
multiple café au lait spots and
neurofibromas scattered all over her body,
being more marked over the trunk area
(Figure 1). Axillary freckles were present
and slit lamp examination of eyes also
revealed multiple Lisch nodules. She also
gave history of similar skin lesions in one
of her four siblings. There were few
excoriation marks over lower back.
Systemic examination was unremarkable
and laboratory investigations (blood
complete picture, liver function tests, renal
function tests, chest x-ray and ultrasound
abdomen) did not reveal any abnormality.
Her pruritus was assumed to be associated
205
Journal of Pakistan Association of Dermatologists 2003; 13: 204-207.
Figure 1 Multiple neurofibromas and café au
lait spots dispersed over whole back of the
patient
with existing neurofibromatosis. She was
given
various
combinations
of
antihistamines along with topical steroidal
and soothing preparations but she did not
show satisfactory response to these
regimens. It was only when ketotifen was
added, she started showing marked
improvement in her symptoms and finally
she was well maintained with ketotifen
alone.
Discussion
NF-1 is a disorder with variable
phenotypic expression. Some patients may
primarily have cutaneous expression,
while others may have life-threatening or
severely
disfiguring
complications.
Pruritus is a rare symptom that can be seen
in NF-1. The possible cause of itching is
histamine and other mediators liberated
from mast cells, as these cells are present
in large number in the skin of NF-1
patients.8,11 The beneficial effects of
antihistamines and mast cell stabilizing
agent in relieving this symptom has been
reported earlier.12,13 The patient described
here had the disease for decades, but she
was not concerned much about the
stigmata of the disease as she never had
206
any symptom related to the pre-existing
characteristic skin lesions of the disease.
She only became worried when she started
having severe itching especially over sites,
where skin lesions of NF-1 were more
concentrated. No other cause of her
pruritus
could
be
elicited.
Unresponsiveness to various combinations
of antihistamines and prompt relief of
itching with ketotifen (a mast cell
stabilizer) gives evidence about the
etiological role of mast cell in producing
symptoms (e.g. pruritus) in patients of NF1. A relatively large number of mast cells
are seen in and around skin tumours
(neurofibromas) in NF-1 patients.11 It is
assumed that these have a pathogenic role
in formation and growth of tumour,
because some of the mast cell mediators
are known to act as growth factors.
Moreover, a mast cell stabilizer, ketotifen
has been shown to decrease neurofibroma
growth, pruritus, pain and tenderness.12,13
Conclusion
NF1 is an extremely variable disorder that
can be compounded by a broad spectrum
of manifestations and pruritus is one of
these rare presentations.
References
1.
2.
3.
4.
5.
Morse RP. Neurofibromatosis type 1.
Arch Neurol 1999; 56: 364-5.
Neurofibromatosis. Conference statement.
National Institutes of Health Consensus
Development Conference. Arch Neurol
1988; 45: 575-8
Karnes PS. Neurofibromatosis: a common
neurocutaneous disorder. Mayo Clin Proc
1998; 73:1071-6.
von Recklinghausen FD. Ueber die
Multiplen Fibrome der Haut and Ihre
Beziehung zu den Multiplen Neuromen.
Berlin: Festschrift fur Rudolf Virchow;
1882.
Barker D, Wright E, Nguyen K et al. Gene
for von Recklinghausen neurofibromatosis
Neurofibromatosis type 1 with generalized pruritus
6.
7.
8.
9.
is in the pericentromeric region of
chromosome 17. Science 1987; 236: 11003
Gutmann DH: Recent insights into
neurofibromatosis type 1: clear genetic
progress. Arch Neurol 1998; 55: 778-80
Lazaro C, Ravella A, Gaona A et al.
Neurofibromatosis type 1 due to germ line
mosaicism in a clinically normal father. N
Engl J Med 1994; 331: 1403-7.
Riccardi VM, ed. Neurofibromatosis:
phenotype,
natural
history,
and
pathogenesis, 2nd edn. Baltimore: John
Hopkins University Press; 1992.
Hope DJ, Mulvihill JJ. Malignancy in
neurofibromatosis. Adv Neurol 1981; 29:
33-56.
A.U. Bari et al.
10. Gutmann DH, Aylsworth A, Carey JC et
al. The diagnostic evaluation and
multidisciplinary
management
of
neurofibromatosis types 1 and 2. JAMA
1997; 278: 51-7.
11. Nurnberger M, Moll I. Semiquantitative
aspect of mast cells and in neurofibromas
of neurofibromatosis type 1 and 5.
Dermatology 1994; 188: 296-9.
12. Riccardi VM. Mast cell stabilization to
decrease
neurofibroma
growth.
Preliminary experience with ketotifen.
Arch Dermatol 1988; 123: 1011-6.
13. Riccardi VM. A controlled multiphase
trial
of
ketotifen
to
minimize
neurofibroma-associated pain and itching.
Arch Dermatol 1993; 129: 577-81.
207
Journal of Pakistan Association of Dermatologists 2003; 13: 208-210.
Case report
Atrophoderma
vermiculata:
disfiguring condition
A
rare
Arfan ul Bari
PAF Hospital, Sargodha.
Abstract Atrophoderma vermiculata is a rare genodermatosis with usual onset in childhood,
characterized by a “honey-combed” reticular atrophy of the cheeks. The course is
generally slow, with progressive worsening. We report a young male with multiple,
ugly looking, worm eaten scars on his cheeks who partially responded to chemical
peeling with trichloracetic acid 35% solution and topical application of 0.05% tretinoin
cream. A brief review of the disorder is also given.
Key words
Atrophoderma vermiculata, keratosis pilaris atrophicans, ulerythema ophryogenes
Introduction
Atrophoderma vermiculata is a rare
genodermatosis that presents as an
inflammatory follicular atrophy. The
morphologic hallmark is a “worm-eaten”
or “honey-combed” reticular atrophy of
the skin typically localized at the cheeks,
preauricular regions, and temples.1,2
Rarely, patients may experience the
characteristic lesions on the extensor
surfaces of the arms and legs. In addition
to the follicular atrophic scars, generalized
facial erythema, sparse open and closed
comedones, and milia can be found.
Associated cutaneous and visceral
abnormalities can occur. The underlying
pathologic defect appears to be an
abnormal
keratinization
of
the
3
pilosebaceous unit. Possible genetic
defect is supposed to be the deletion of
chromosome
18p.4 This condition
generally has its onset in childhood,
although some cases arising during
puberty or adulthood have been seen.5
Address for Correspondence
Squadron Leader Dr. Arfan ul Bari,
Consultant Dermatologist,
PAF Hospital, Sargodha.
E mail: albariul@yahoo.com
208
Histologically, in the early phase,
pilosebaceous follicles filled with keratotic
plugs
and
a
mild
perifollicular
inflammatory infiltrate are observed.
Cystic dilatation of the hair follicles may
be evident on the cheeks. Later, atrophy of
both hair follicles and sebaceous glands, as
well as dermal fibrosis, may appear. The
course is generally one of slow
progressive worsening; however, instances
of spontaneous regression have also been
reported. Primarily a cosmetic problem,
therapy for this condition is aimed at
reassurance, genetic counseling, and
dermabrasion where appropriate. Other
options include cryotherapy, ultraviolet
light radiation, and several topical
medications. Carbon dioxide (CO2) and
585nm pulsed dye lasers have also
recently been used with success.6-8 A case
of successful induction of remission in the
inflammatory component of the disease
has also been reported in the literature,
following a prolonged course of
isotretinoin.9
Atrophoderma vermiculata: A rare disfiguring condition
Arfan ul Bari
folliculitis
Case history
A 25-year-old male reported with history
of multiple disfiguring scars over his right
cheek since childhood. During last one
year, he noticed two painless nodular
swellings and a few small scars over left
cheek. There was no history of any other
preceding or concomitant cutaneous
disorders like acne, folliculitis, small pox
or chicken pox. None of his family
members had similar lesions. On
dermatological examination he was found
to have multiple 1-3 mm sized pitted and
ice picks scars with ridges producing a
reticulated honeycomb appearance on
right cheek (Figure 1). Sparse open and
closed comedones were present but
inflammatory acne lesions, milia and
facial erythema were notably absent. Two
nodulocystic swellings along with a few
small scars and closed comedones were
also seen over left cheek (Figure 2). Due
to peculiar worm eaten appearance, typical
site of involvement and characteristic
morphology of the lesions he was
clinically diagnosed as a case of
atrophoderma vermiculatum. Nodular
swellings on right side were excised and
on histology these turned out to be
sebaceous cysts. He was explained the
nature of his disease and was advised
fortnightly
chemical
peeling
with
trichloracetic acid 35% solution and daily
topical application of 0.05% tretinoin
cream. He showed some response to this
treatment regimen but then was lost to
follow up.
Discussion
Atrophoderma
vermiculatum
is
a
condition that also has been called
atrophoderma
reticulatum,
acne
vermoulante, folliculitis ulerythematosa
reticulata,
and
honeycomb
Figure 1 Multiple pitted and ice picks scars
along with few scattered comedones
Figure 2 Left side of the face showing two
sebaceous cysts few scars and closed
comedones
atrophy. It has been classified as one of the
four conditions that present with keratosis
pilaris atrophicans (KPA), which is
characterized by follicular hyperkeratosis,
inflammation, and scars. The other entities
that show KPA are keratosis pilaris
atrophicans
faciei,
ulerythema
ophryogenes and folliculitis spinulosa
decalvans.10 Some authors believe these
entities are different presentations of the
same
condition.
Atrophoderma
vermiculata is characterized by erythema
and reticulate atrophic scarring of the face
and may also be called as a follicular
syndrome with inflammation and atrophy.
The other variants of this syndrome can be
distinguished
from
atrophoderma
vermiculata by location, degree of
209
Journal of Pakistan Association of Dermatologists 2003; 13: 208-210.
inflammation, mode of inheritance, and
histologic pattern. Each of these diseases
usually manifests in infancy or childhood
and runs a chronic course with only rare
spontaneous regression seen. Their
common pathologic features are follicular
dilation, hyperkeratosis, and ultimate
follicular destruction. Patients with
atrophoderma vermiculata are often
psychologically affected by their obvious
facial lesions and thus are compelled to
seek cosmetically effective treatment.
Because there are no curative therapeutic
modalities available, palliative treatment
has been attempted with topical steroids,
tretinoin creams, and systemic retinoids.
More aggressive treatment modalities
include cryotherapy, dermabrasion, and
laser therapy. The patient we described
here was a typical case of atrophoderma
vermiculatum who was psychologically
much disturbed due to obvious
disfigurement of his face. Because of nonavailability of suitable laser in the town
and financial constraint of the patient, he
was offered medium depth chemical
peeling and topical tretinoin, to which, he
partially responded. We think appropriate
laser therapy or oral isotretinoin would
have given him better results.
References
210
1.
Rozum LT, Mehregan AH, Johnson SAM.
Folliculitis ulerythematosus reticulata: a
case with unilateral lesion. Arch Dermatol
1972; 106: 388-9.
2. Frosch PJ, Bumage MR, SchusterPavlovic C et al. Atrophoderma
vermiculatum: case reports and review. J
Am Acad Dermatol 1988; 18: 538-42.
3. Barron DR, Hirsch AL, Buchbinder L, et
al. Folliculitis ulerythematosus reticulata:
a report of four cases and brief review of
the literature. Pediatr Dermatol 1987; 4:
85-9.
4. Nazarenko SA, Ostroverkhova NV,
Vasiljeva EO et al. Keratosis pilaris and
ulerythema ophryogenes associated with
an 18p deletion caused by a Y/18
translocation. Am J Med Genet 1999; 85:
179-82.
5. Ellis
JP.
Familial
atrophoderma
vermiculatum: case report. Br J Dermatol
1980;103: 57-8.
6. Handrick C, Alster TS. Laser treatment of
atrophoderma vermiculata. J Am Acad
Dermatol 2001; 44: 693-5.
7. Clark SM, Mills CM, Lanigan SW.
Treatment of keratosis pilaris atrophicans
with the pulsed tunable dye laser. J Cutan
Laser Ther 2000; 2: 151-6.
8. Chui CT, Berger TG, Price VH, Zachary
CB. Recalcitrant scarring follicular
disorders treated by laser-assisted hair
removal: a preliminary report. Dermatol
Surg 1999; 25: 34-7.
9. Weightman W. A case of atrophoderma
vermiculatum responding to isotretinoin.
Clin Exp Dermatol 1998; 23: 89-91.
10. Oranje AP, van Osch LD, Oosterwijk JC.
Keratosis
pilaris
atrophicans:
one
heterogenous disease or a symptom in
different clinical entities? Arch Dermatol
1994; 130: 500-2.
Journal of Pakistan Association of Dermatologists 2003; 13: 211-213.
Quiz
What are these pIaques?
Amor Khachemoune, Shahbaz A Janjua
Ayza Skin and Research Centre, Lalamusa
An 8-year-old, otherwise healthy, girl
presented with 1-month history of an
eruption of mildly pruritic, rough plaques
over the skin of her elbows, knees and
upper lateral thighs. Her personal and
family
medical
histories
were
unremarkable; particularly there was no
history of atopic diathesis. She denied the
use of any medication in the recent past.
On physical examination, there were
multiple, symmetrically distributed, 2 cm
to 6 cm round to oval plaques comprised
of multiple, grouped, monomorphous,
follicular, keratotic, skin colored, 1 mm to
2 mm papules affecting the skin over her
elbows (Figure 1), knees and upper lateral
thighs. Each papule was bearing a central,
protruding, thorny, hair-like, 1-mm
keratotic spine (Figure 2). Although, the
papules were grouped into plaques,
discrete follicular, keratotic papules were
also present in the vicinity of the plaques.
The lesions were not biopsied, as the
consent could not be obtained from her
parents.
Figure 1
Figure 2
Address for Correspondence
Dr. Shahbaz A. Janjua, MD,
Ayza Skin and research Centre, Lalamusa.
E mail: dr_janjua@hotmail.com
211
What are these plaques?
Diagnosis
Lichen spinulosus
Lichen spinulosus (LS) is a rare,
idiopathic, follicular, keratotic dermatosis
occurring mostly in children, adolescents
and young adults. The male to female ratio
is 2:3.1 No race predilection or seasonal
variations have been noted. This condition
was first recorded in the literature by
Crocker in 1883. He named it “lichen
pilaris seu spinulosus.” Since then, there
were few case reports until Friedman
recorded 35 cases in the Philippines.1
Characterized by an acute development of
symmetrically
distributed
follicular,
keratotic papules that are sharply grouped
in round to oval plaques varying in size
from 2 cm to 6 cm, LS can appear on the
extensor surfaces of elbows and knees, the
neck, buttocks, abdomen and the
trochanteric regions. Very rarely, the
eruption can be generalized. A typical LS
papule is 1 mm to 3 mm in diameter with
skin-colored conical projection and a
central 1-mm to 2-mm keratotic spine. The
eruption may be asymptomatic or only
mildly pruritic. The lesions may spring up
simultaneously or in crops.
Etiology and pathology
Although the exact etiology of LS is
uncertain, it has been described as a
follicular reaction pattern to various
environmental agents such as infections,
toxins, chemicals and drugs.2 It has been
attributed to atopy,3 but clear evidence is
lacking. Because lesions occur on pressure
areas, trauma has been considered a cause,
although clinical findings do not suggest
koebnerization.1 LS is also described in
patients with HIV infection,3,4 and in
association with Crohn’s disease.5 In short,
LS remains an idiopathic dermatosis.
212
Amor Khachemoune & S.A Janjua.
On histopathology, LS lesions show a
central, infundibular, keratotic plug
dilating the follicle and protruding above
the
epidermal
surface.
Follicular
hyperkeratosis,
parakeratosis
and
acanthosis may be present. Dermal
perifollicular
and
perivascular
lymphocytic infiltrate is also seen under
light microscopy. It is pertinent to note
that
LS
is
histopathologically
indistinguishable from keratosis pilaris.
Differential diagnosis
Differential diagnosis of LS is broad and
includes
other
follicular
keratotic
dermatoses such as the following
dermatoses:
• Keratosis pilaris may closely mimic LS,
but is a common dermatosis, of slow
onset, appearing most commonly in the
first decades of life. Seasonal variation
with winter exacerbation is another
differentiating point. Morphologically, it is
differentiated from LS by the even but
diffuse distribution of smaller, keratotic
follicular papules, each with a protruding
or embedded central hair, occurring
commonly on the extensor surfaces of
proximal limbs. There are many clinical
subtypes with keratosis pilaris alba being
the most common and differentiated from
keratosis pilaris rubra, another subtype, by
the absence of perifollicular erythema.
There is a frequent association of keratosis
pilaris with ichthyosis vulgaris.
• Pityriasis rubra pilaris may be familial
or acquired with acute onset but is
differentiated from LS by characteristic
reddish-orange or salmon-colored scaling,
palmoplantar keratoderma and keratotic
follicular papules on the dorsal surfaces of
the proximal phalanges, elbows and
wrists.
Journal of Pakistan Association of Dermatologists 2003; 13: 211-213.
• Darier’s disease is an autosomal
dominant genodermatosis, characterized
by the symmetrical appearance of
follicular keratotic, skin-colored papules
on the seborrheic areas. The papules,
which are pruritic, ultimately turn
yellowish brown and greasy. Palms are
commonly involved and the nails undergo
changes consisting of red and white
longitudinal stripes, ridging and nicking at
the distal ends, and are often
pathognomonic. Mutations in the gene
ATP2A2 (Darier’s gene) have been
described to be responsible for the disease.
The affected gene encodes for the calcium
pump, so the mutation alters cytosolic
calcium levels, resulting in disruption of
the desmosomes and keratin filaments.
• Phrynoderma is described as cutaneous
manifestations of vitamin A deficiency
characterized by follicular hyperkeratosis
affecting anterior thighs and posterior
arms due to keratinization of the hair
follicles giving the general appearance of
“toad skin.” Vitamin A deficiency could
be primary, mainly due to inadequate
intake, or less commonly could be
secondary to interruption with the
absorption, storage and transport of the
vitamin as in celiac disease, cirrhosis of
the liver and surgical resection of the
duodenum. Children with chronic
malnutrition are especially prone to the
effects of the hypovitaminosis A. Other
manifestations include night blindness,
xerophthalmia,
keratomalacia
and
keratinization of the mucosal epithelia of
the gastrointestinal and urinary tracts. Eye
changes are the earliest signs detected and
consist of retinal dysfunction and xerosis
of the cornea and conjunctiva. Advanced
disease is characterized by the presence of
superficial foamy patches of epithelial
debris on the bulbar conjunctiva; these are
commonly called Bitot’s spots.
Treating LS
Lichen spinulosus does not usually
spontaneously remit and may persist for
several years, if left untreated. However,
the lesions may involute gradually over
several years. Treatment aims to address
the
cosmetically
unappealing
disfigurement caused by the disease. It
includes topical application of keratolytic
agents including salicylic acid, lactic acid
and
urea
available
in
different
formulations and concentrations. In one
study, overnight application of 6%
salicylic acid gel under occlusion for 2
weeks and without occlusion for 8 weeks
resulted in significant reduction of the
keratotic lesions.6 Topical steroids may be
added if pruritus is also present. In another
study, treatment with 12% lactic acid
lotion and triamcinolone in a moisturizing
base also resulted in significant
improvement.3 Topical retinoids have no
role in treating LS.
References
1.
2.
3.
4.
5.
6.
Friedman
SJ.
Lichen
spinulosus.
Clinicopathologic review of thirty-five
cases. J Am Acad Dermatol 1990; 22: 2614.
Becker SW. Lichen spinulosus following
intradermal application of diphtheria
toxin. Arch Dermatol Syph 1930; 21:83940.
Strickling WA, Norton SA. Spiny eruption
on the neck. Diagnosis: lichen spinulosus
(LS). Arch Dermatol 2000; 136: 1165-70.
Cohen SJ, Dicken CH. Generalized lichen
spinulosus in an HIV positive man. J Am
Acad Dermatol 1991; 25:116-8.
Kano Y, Orihara M, Yagita A, Shiohara T.
Lichen spinulosus in a patient with
Crohn's disease. Int J Dermatol 1995; 34:
670-1.
Maiocco KJ, Miller OF. Lichen
spinulosus: response to therapy. Cutis
1976; 17: 294-9.
213
Journal of Pakistan Association of Dermatologists 2003; 13: 214
News
National events
2004
December 9-12, 2004
Silver Jubilee Conference of Pakistan
Association of Dermatologists, Karachi.
Organizing Chairman: Dr. Khurshid H.
Alvi, Suite No. 11, 3rd Floor, Taj Medical
Complex, M.A. Jinnah Road, Karachi,
74400 Pakistan
Tel: +92 21 7789666
Fax: +92 21 7789677
E-mail: silver@pad.org.pk
info@pad.org.pk
Website: www.pad.org.pk
281st Free Skin Camp, Piryaloi City,
District Khairpur Mirs 5th October,
2003.
In collaboration with Al-Makhdoom
Naujawan Samajee Taanzeem, Piryaloi,
Welfare Association for Dermatological
Patients (WADeP) arranged its 281st Free
Skin Camp at Government Boys Primary
School No. 1, Piryaloi on 5th October,
2003. A total of 1589 patients from
different areas of Khairpur, Shikarpur,
Sukkur, Ghotki, Larkana and Rahimyar
Khan districts visited the camp. Patients
were provided free consultation and
medicaments.
282nd Free Skin Camp, Badin City,
District Badin 19th October, 2003.
In collaboration with Sindh Graduates
Association, Badin Branch, Welfare
Association for Dermatological Patients
(WADeP) arranged its 282nd Free Skin
Camp at Government Main Primary
school, Badin City on 19th October, 2003.
A total of 1266 patients from different
areas of Thatta, Thar, Badin, Mirpur Khas,
Hyderabad and Sanghar districts visited
the camp. Patients were provided free
consultation and medicaments.
214
International events
2004
February 6-11
American Academy of Dermatology 62nd
Annual Meeting, Washington, DC
For more information contact:
American Academy of Dermatology, 930
E Woodfield Rd, Schaumberg, IL
60173/847-330-0230; fax 847-330-1090.
E-mail: rescalante@aad.org
May 15-18
ISD Regional Meeting
Dermatology and Dermato-Cosmetology
Congress
Secretariat Office
c/o Skin and Allergy Centre
540 3rd Floor Mercury Tower
Ploenchit Road
Patumwan, Bangkok 10330
Thailand
Tel: 662 658 5885
Fax: 662 658 5884 or 662 433 7923
E-mail: thadapiam@thaicosderm.org
May 19-22
IX ISD International Congress on
Dermatology, Beijing, China
Contact: International Congress Secretariat
Tel: +86 10 6524 9989 ext 1606
Fax: + 86 10 6512 3754
E-mail: icd2004@chinamed.com.cn
November 17-21
13th Congress of the European Academy
of Dermatology and Venereology
Contact: Torello M. Lotti, Florence, Italy
E-mail: president@eadv2004.org or
info@eadv2004.org
2005
October 12-15
European Academy of Dermatology and
Venereology Congress (EADV)
London, UK
Contact: Marilyn Benham
Tel: 020 7383 0266
E-mail: eadv@bad.org.uk
www.eadv.org
Journal of Pakistan Association of Dermatologists 2003; 13: 215-216.
Author Index, Volume 13, 2003
ABBAS, M. see BARI, A.U.
AGHA, H. see BARI, A.U.
AHMAD, A. see BADER, E.
AHMAD, N. see BAJWA, U.M.
AHMAD, S., AMAN, S., HUSSAIN, I.,
HAROON, T.S. A clinico-etiological study of
toe web fungal infection in Lahore 62
AHMAD, T.J. see RANI, Z.
AHMAD, T.J., RASHID, T., RANI, Z.
Modifications in punch grafting 146
AHMAD, T.J., RASHID, T., RANI, Z.,
HAROON, T.S. Autologous skin punch
grafting in localized, fixed vitiligo 114
AHMAD, T.M. see AMAN, I.
AHMED, A. see IRSHAD, W.
AHMED, I., ANSARI, M., MALICK, K. An
audit of dermatoses at Baqai Institute of Skin
Diseases, Karachi 117
AHMED, I., ANSARI, M., MALICK, K.
Childhood eczema: a comparative analysis 164
AHMED, I., WAHID, Z., AHMED, Z.
Chronic urticaria: frequency of anti-HCV
antibodies 179
AHMED, I., WAHID, Z., AHMED, Z.
Vitiligo: Effect of levamisole pulse therapy 13
AHMED, Z. see AHMED, I.
AKHTAR, S.J. Comparison of 4 and 40
milliseconds pulse durations for Nd: YAG hair
removal laser 193
AMAN, I., AMAN, S., AHMAD, T.M. A
family with xeroderma pigmentosumCockayne syndrome complex 153
AMAN, S. see AHMAD, S.
AMAN, S. see AMAN, I.
AMAN, S., HUSSAIN, I., HAROON, T.S.
Successful treatment of tinea capitis due to
Microsporum canis with griseofulvin 123
ANSARI, M. see AHMED, I.
ANWAR, M see IRSHAD, W.
ANWAR, M., RIZWAN, N., SHEIKH, N.R.
Frequency and complications of irrational use
of topical steroids in dermatophytosis 193
ARIF, S., MAAN, M.A., IQBAL, A.J.,
SHEIKH, M.S. Irritation with toilet soaps 17
ASAD, F., PAL, S.S. An erythematous nodule
on outer aspect of arm in a female with breast
carcinoma 97
ASAD, F., PAL, S.S. An erythematous plaque
on the breast 157
ASAD, F., PAL, S.S., HAROON, T.S.
Histological spectrum and clinicopathological
correlation of cutaneous granulomas: our
experience at Mayo Hospital, Lahore, Pakistan
193
AZIZ, F. see KHAN, Y.
BADER, E., AHMAD, A. FNAC in
granulomatous and malignant skin conditions
BADER, E., SHEIKH, R. Efficacy of
itraconazole
in
the
management
of
dermatological fungal infections 193
BAJWA, U.M., AHMAD, N. Non Hodgkin’s
lymphoma 193
BARI, A.U. Atrophoderma vermiculata: A rare
disfiguring condition 208
BARI, A.U. see RAHMAN, S.,B.
BARI, A.U., ABBAS, M., RAHMAN, S.B.
Acrodermatitis enteropathica in three siblings.
148
BARI, A.U., AGHA, H., RAHMAN, S.B.
Neurofibromatosis type 1 with generalized
pruritus 193
BARI,
A.U.,
RAHMAN,
S.B.
Neurofibromatosis and Caroli's disease - an
extremely rare combination 193
BARI, A.U., RAHMAN, S.B. Zinc: An
overview and therapeutic uses in dermatology
130
BARI, A.U., RAHMAN, S.B. Zosteriform
lichen planus: a new variant of a common
disorder 193
BHUTTO, A.M., SOLANGI, A.H., KUMAR,
D. Xeroderma pigmentosum in Larkana,
Pakistan. Clinical and histopathological
observations 193
BOKHARI, S.M.A. see KHALID, T.
BUTT, A.Q. see TAMEEZ-UD-DEEN
DAR, N.R. see DILNAWAZ, M.
DILNAWAZ, M., DAR, N.R. Histiocytoses
184
DILNAWAZ, M., DAR, N.R., MUSHTAQ, S.
Cutaneous lymphomas and lymphocytic
infiltrates: A clinicopathological correlation
and therapeutic options 29
GHANI, R. see KHAN, Y.
HAFEEZ, J. see RAHMAN, S.B.
HAMEED, A. Useful electrode modification
for electro surgery 202
HAROON, T.S. see AHMAD, S., AHMAD,
T.J., AMAN, S., ASAD, F., MAHMOOD, T.,
RANI, Z., SHAHEEN, J.A., SHAMSUDDIN,
S., SHEHZAD, A.
HUSSAIN, I. Reporting of clinical trials.
Should we adopt CONSORT statement? 52
HUSSAIN, I. see AHMAD, S., AMAN, S.,
MUZAFFAR, F., RANI, Z., SHAHEEN, J.A.
IQBAL, A.J. see ARIF, S.
IRSHAD, W., AHMED, A., ANWAR, M
Etiologic break up in cases of erythroderma in
our community as assessed by clinical and
histopathological evaluation 193
215
Author Index
JAHANGIR, M. Are young medical graduates
equipped with required minimum dermatology
knowledge? 193
JANJUA, S.A. see KHACHEMOUNE, A.
KHACHEMOUNE, A., JANJUA, S.A. What
are these plaques? 211
KHALID, T., MAAN, A., BOKHARI, S.M.A.
Contact reactions in hospital workers 193
KHAN, A.A. see RAHMAN, S.B.
KHAN, F.A., NAKIB, K.A., QUABA, A.A.R.
Microsclerotherapy as a modality of treatment
in superficial venous malformations 72
KHAN, Y. Botox in dermatology, 1
KHAN, Y., AZIZ, F., YAZDANI, I. Telogen
effluvium in a patient on haemodialysis. A
case report and review of literature 43
KHAN, Y., GHANI, R., GHANI, S.,
THAWERANI, H. Lipoid proteinosis: A case
report with review of literature 45
KHURSHID, K. see SHEHZAD, A.
KUMAR, D. see BHUTTO, A.M.
KUNDI, Z.U. see QURESHI, R.
MAAN, A. see KHALID, T.
MAAN, M.A. see ARIF, S.
MAHMOOD, T. see SHAHEEN, J.A.
MAHMOOD, T., HAROON, T.S. Patterns of
direct immunofluorescence in sub-epidermal
autoimmune bullous diseases of skin in
Lahore, Pakistan 67
MALICK, K. see AHMED, I.
MALIK, F. see QURESHI, R.
MANN, M.A. Irritation with toilet soaps 193
MASHHOOD, A.H. Sensitivity of various
laboratory investigations in the diagnosis of
Old World cutaneous leishmaniasis 193
MEHMOOD, K. Beaded papular epidermal
hyperplasia of neck 193
MEHMOOD, K. Retinitis pigmentosa like oral
pigmentation in association with retinitis
pigmentosa 193
MUHAMMAD, S. Cutaneous manifestations
of thyrotoxicosis in 100 hospital admitted
cases 21
MUSHTAQ, S. see DILNAWAZ, M.
MUZAFFAR, F. Non-Langerhans cell
histiocytoses 161
MUZAFFAR, F., HUSSAIN, I. Cockayne
syndrome. An update 135
NAKIB, K.A. see KHAN, F.A.
NAQQASH, S. see TAMEEZ-UD-DEEN
PAL, S.S. see ASAD, F.
PAL, S.S. see SHEHZAD, A.
QUABA, A.A.R. see KHAN, F.A.
QURESHI, R. Treatment of depressed scar
marks without surgery 193
QURESHI, R., MALIK, F., KUNDI, Z.U.
Chronic mucocutaneous candidiasis - a fatal
disease 193
RAHMAN, A., SHEIKH, Z.I., RAHMAN,
S.B.
Secondary
syphilis
mimicking
216
palmoplantar pustular psoriasis an unusual
clinical presentation 193
RAHMAN, S.B. see BARI, A.U., RAHMAN,
A.
RAHMAN, S.B., BARI, A.U., KHAN, A.A. A
new focus of cutaneous leishmaniasis in
Pakistan 3
RAHMAN, S.B., BARI, A.U., RASHID, H.U.
Role of serodiagnosis in cutaneous
leishmaniasis 171
RAHMAN, S.B., HAFEEZ, J. Deep mycosis
following immunosuppression for pemphigus
vulgaris 193
RANI, Z. see AHMAD, T.J.
RANI, Z. Vitiligo. From Babechi to lasers 112
RANI, Z., AHMAD, T.J., HUSSAIN, I.
Vohwinkel syndrome: Case report and review
of literature 92
RANI, Z., HUSSAIN, I. Immunofluorescence
in immunobullous diseases 76
RANI, Z., HUSSAIN, I., HAROON, T.S.
Effect of contact dermatitis on dermatology
life quality index 193
RANI, Z., RASHID, T., HUSSAIN, I.
Erythema annulare centrifugum in association
with hypothyroidism: a case report 89
RASHID, H.U. see RAHMAN, S.B.
RASHID, T. Patients’ perceptions about acne.
A questionnaire study in 100 patients with
acne 193
RASHID, T. see AHMAD, T.J.
RASHID, T. see RANI, Z.
RIZWAN, N. see ANWAR, M.
SAEED, H. Effect of LPIR alexandrite laser on
Asian skin & safety of laser in pregnancy 193
SAVANT, S.S. Cryosurgery 35
SHAHEEN, J.A., HAROON, T.S., HUSSAIN,
I., MAHMOOD, T. Evaluation of sensitivity of
Tzanck smear in pemphigus 175
SHAMSUDDIN, S., HAROON, T.S.
Comparative study of psoralen-UVB vs. DYEalone therapy in the treatment of psoriasis 55
SHEHZAD, A., KHURSHID, K., PAL, S.S.,
HAROON, T.S. Topical calcipotriol vs. oral
psoralen-UVA
(PUVA)
and
topical
calcipotriol in the treatment of vitiligo in typeIV skin 193
SHEIKH, M.S. see ARIF, S.
SHEIKH, N.R. see ANWAR, M.
SHEIKH, R. see BADER, E.
SHEIKH, Z.I. see RAHMAN, A.
SOLANGI, A.H. see BHUTTO, A.M.
TAMEEZ-UD-DEEN, NAQQASH, S., BUTT,
A.Q. Lichen planus and hepatitis C virus
infection: An epidemiologic study 127
THAWERANI, H. see KHAN, Y.
WAHID, Z. see AHMED, I.
YAZDANI, I. see KHAN, Y.
ZAHID, M.A. Bowen's disease of male breast
- A rare entity 193
Journal of Pakistan Association of Dermatologists 2003; 13: 217-220.
Subject Index, Volume 13, 2003
acrodermatitis enteropathica
and zinc 127
in three siblings (case report) 148
actinic reticuloid
cutaneous lymphomas and
lymphocytic infiltrates 29
alpha-hydroxy acids
in melasma 21
lymphoma, angiotropic
cutaneous lymphomas and
lymphocytic infiltrates 29
atrophoderma vermiculatum (case report) 208
azelaic acid ,in melasma 21
bacterial infections
an audit of dermatoses at Baqai
Institute 117
Baker’s formula
in melasma 21
Baqai Institute, skin diseases
an audit of dermatoses at Baqai
Institute 117
benign cephalic histiocytosis 184
beta-hydroxy acids, in melasma 21
botox
in dermatology (editorial) 1
Bowen’s disease, of male breast (abstract) 193
bullous diseases
patterns of direct
immunofluorescence in subepidermal
autoimmune bullous diseases 67
bullous disorders
an audit of dermatoses at Baqai
Institute 117
bullous pemphigoid
immunofluorescence 76
acne, patients’ perceptions (abstract) 193
calcipotriol
topical vs. topical calcipotriol plus
psoralen-UVA in vitiligo (abstract)
193
Candida
a clinico-etiological study of toe-web
fungal infection 62
candidiasis, mucocutaneous 193
carbon dioxide snow
cryosurgery 35
chemical peel
in melasma 21
cicatricial pemphogoid
immunofluorescence 76
clear cell hidradenoma (quiz) 97
Cockayne syndrome (review) 135
CONSORT statement 52
contact
dermatitis and quality of life 193
reactions in hospital workers 193
cryosurgery 35
cutaneous manifestations, in thyrotoxicosis 17
cutaneous plasmacytoma
cutaneous lymphomas and
lymphocytic infiltrates 29
cytology, fine needle aspiration
in granulomatous and malignant
disease 193
dermatitis herpetiformis
immunofluorescence 76
dermatofibroma
dermatology
knowledge, medical graduates
dermatophytosis, irrational use of topical
steroids (abstract) 193
diffuse plane xanthomatosis 184
direct immunofluorescence (DIF)
patterns in subepidermal autoimmune
bullous diseases 67
drug eruptions
an audit of dermatoses at Baqai
Institute 117
eczema
an audit of dermatoses at Baqai
Institute 117
atopic 117
childhood 117
infective 117
nummular 117
seborrheic 117
xerotic 117
electro surgery, electrode modification 202
epidemiology, skin diseases
an audit of dermatoses at Baqai
Institute 117
lichen planus and hepatitis C 127
epidermal hyperplasia, beaded, of neck
(abstract) 193
epidermolysis bullosa acquisita,
immunofluorescence 76
Epidermophyton floccosum
a clinico-etiological study of toe-web
fungal infection 62
erythema annulare centrifugum
in association with hypothyroidism
(case report) 89
erythroderma
etiologic break up, clinical,
histopathologic features 193
follicular mucinosis
cutaneous lymphomas and
lymphocytic infiltrates 29
fungal infections
an audit of dermatoses at Baqai
Institute 117
generalized eruptive histiocytoma 184
217
Subject Index
Gordon’s formula
in melasma 21
granulomas, cutaneous
histological spectrum and
clinicopathological correlation 193
granulomatous slack skin disease
cutaneous lymphomas and
lymphocytic infiltrates 29
griseofulvin
treatment in tinea capitis 123
HCV
and chronic urticaria 179
hepatitis C
with lichen planus 127
histiocytoses (review)
Langerhans cell 184
malignant 184
non-Langerhans cell 184
histiocytosis
familial hemophagocytic
lymphohistiocytosis 184
familial sea blue 184
sinus histiocytosis with massive
lymphadenopathy 184
virus-associated hemophagocytic
syndrome 184
history
Journal of Pakistan Association of
Dermatologists 48
hospital-based prevalence
an audit of dermatoses at Baqai
Institute 117
hydroquinone, in melasma 21
ICMJE
uniform requirements for manuscripts
submitted to biomedical journals 99
immunocytoma
cutaneous lymphomas and
lymphocytic infiltrates 29
immunofluorescence
in immunobullous diseases 76
itraconazole
efficacy in the management of fungal
infections 193
Jessner’s lymphocytic infiltrate
cutaneous lymphomas and
lymphocytic infiltrates 29
Jessner’s solution
in melasma 21
juvenile xanthogranuloma 193
Karachi, Pakistan
an audit of dermatoses at Baqai
Institute 117
kojic acid, in melasma 21
Lahore
patterns of direct
immunofluorescence in subepidermal
autoimmune bullous diseases 67
lasers
alexandrite for hair removal, effect
218
and safety in pregnancy 193
in melasma 21
Nd:YAG, hair removal, effect of
pulse duration 193
leishmaniasis
an audit of dermatoses at Baqai
Institute 117
leishmaniasis, cutaneous
a new focus in Pakistan 3
enzyme-linked immunosorbent assay
(ELISA) 171
immunofluorescence antibody test
(IFAT) 171
indirect hemagglutination test (IHT)
171
sensitivity of various diagnostic
methods (abstract) 193
serodiagnosis 171
serological tests 171
leukemia, monocytic 184
levamisole
in vitiligo 7
lichen planus
and hepatitis C 127
zosteriform variant 193
lichen spinulosus, what are these plaques? 211
linear IgA disease, immunofluorescence 76
lipoid proteinosis 45
liquid nitrogen, cryosurgery 35
lupus erythematosus, bullous
immunofluorescence 76
lymphocytic infiltrates
clinicopathological correlation and
therapeutic options 29
lymphocytoma cutis
cutaneous lymphomas and
lymphocytic infiltrates 29
lymphoma, cutaneous, B cell
clinicopathological correlation and
therapeutic options 29
lymphoma, cutaneous, T cell (CTCL)
clinicopathological correlation and
therapeutic options 29
true histiocytic 184
non-Hodgkin (abstract) 193
lymphoma, follicular center cells
cutaneous lymphomas and
lymphocytic infiltrates 29
lymphomatoid granulomatosis
cutaneous lymphomas and
lymphocytic infiltrates 29
malakoplakia 184
melasma
an audit of dermatoses at Baqai
Institute 117
overview and update 21
microsclerotherapy
as a modality of treatment in
superficial venous malformations 72
Journal of Pakistan Association of Dermatologists 2003; 13: 217-220.
Microsporum canis
tinea capitis caused by 123
multicentric reticulohistiocytosis 184
mycobacterial infections
an audit of dermatoses at Baqai
Institute 117
mycosis fungoides
cutaneous lymphomas and
lymphocytic infiltrates 29
mycosis, deep, in immunosuppression for
pemphigus 193
N-acetyl-4-s-cysteaminyl phenol
in melasma 21
necrobiotic xanthogranuloma 184
Neill-Dingwall syndrome 135
neurofibromatosis, type 1
and Caroli’s disease 193
with generalized pruritus (case report)
204
nevoid disorders
an audit of dermatoses at Baqai
Institute 117
Paget’s disease
of breast 157
pagetoid reticulosis
cutaneous lymphomas and
lymphocytic infiltrates 29
Pakistan
a new focus of cutaneous
leishmaniasis 3
papulosquamous disorders
an audit of dermatoses at Baqai
Institute 117
parapsoriasis
cutaneous lymphomas and
lymphocytic infiltrates 29
pediculosis
an audit of dermatoses at Baqai
Institute 117
pemphigoid gestationis
immunofluorescence 76
pemphigus
immunofluorescence 76
sensitivity of Tzanck smear 175
phenol, in melasma 21
pigmentary disorders
an audit of dermatoses at Baqai
Institute 117
pityriasis alba 164
pompholyx 164
progeria-like syndrome 135
Progeroid nanism 135
pruritus
an audit of dermatoses at Baqai
Institute 117
generalized in neurofibromatosis
(case report) 204
pseudolymphoma
cutaneous lymphomas and
lymphocytic infiltrates 29
psoralen-UVB
comparative study with UVB-alone in
psoriasis 55
psoriasis
comparative study of psoralen-UVB
vs. UVB-alone 55
punch grafting
autologous, in vitiligo 114
modifications 146
retinitis pigmentosa-like pigmentation
(abstract) 193
Sabouraud’s dextrose agar
a clinico-etiological study of toe-web
fungal infection 62
scabies
an audit of dermatoses at Baqai
Institute 117
scars, depressed
treatment without surgery 193
sebaceous gland diseases
an audit of dermatoses at Baqai
Institute 117
sensitivity
of Tzanck smear in pemphigus 175
Sezary syndrome
cutaneous lymphomas and
lymphocytic infiltrates 29
soaps, irritation with 13
steroids, topical, irrational use in
dermatophytosis (abstract) 193
sunscreen, in melasma 21
syphilis,
secondary, mimicking palmoplantar
pustular psoriasis (abstract) 194
telogen effluvium
with hemodialysis 43
thyrotoxicosis
cutaneous manifestations 17
tinea capitis
an audit of dermatoses at Baqai
Institute 117
treatment with griseofulvin 123
tinea pedis
a clinico-etiological study of toe-web
fungal infection 62
toe web fungal infection
a clinico-etiological study 62
tretinoin, in melasma 21
trichloracetic acid, in melasma 21
Trichophyton rubrum
a clinico-etiological study of toe-web
fungal infection 62
Trichophyton violaceum
a clinico-etiological study of toe-web
fungal infection 62
trichothiodystrophy 135
tumours, cysts
an audit of dermatoses at Baqai
Institute 117
Tzanck smear, sensitivity in pemphigus 175
219
Subject Index
urticaria, chronic
and anti-HCV antibodies 179
UVB, -alone
comparative study with psoralenUVB in psoriasis 55
venous malformations, superficial
microsclerotherapy as a modality of
treatment 72
viral infections
an audit of dermatoses at Baqai
Institute 117
vitiligo
autologous punch grafting in fixed
type 114
effect of levamisole pulse therapy 7
from Babechi to lasers 112
topical vs. topical calcipotriol plus
220
psoralen-UVA in vitiligo (abstract)
193
Vohwinkel’s syndrome (case report) 92
xanthoma disseminatum 184
xeroderma pigmentosum 135
clinical and histopathological
observations in Larkana, Pakistan 193
-Cockayne syndrome complex in a
family (case report) 153
Cockayne syndrome (review) 135
zinc
an overview and therapeutic uses
(review) 130
in acne 127
in acrodermatitis enteropathica 127
Information for Authors
Manuscripts
The JPAD agrees to accept manuscripts prepared in
accordance with the “Uniform Requirements for
Manuscript Submission to the Biomedical Journals”
approved by the International Committee of
Medical Journals Editors. Three copies of all
material for publication should be sent to Dr. Ijaz
Hussain,
Editor,
JPAD,
Department
of
Dermatology, Mayo Hospital, Lahore, e-mail:
dr_ijazhussain@hotmail.com
dr_ijazhussain@yahoo.com
Manuscripts should be printed on one side of paper
only, with a 2.5 cm margin on either side, be
double spaced, and bear the title of the paper, name
and address of each author, together with the name
of the hospital, laboratory or institution where the
work has been carried out. The name and full
address of corresponding author should be given on
the first page. Pages should be numbered. Authors
should keep a copy of the manuscript.
In addition to the hard copy, an exact copy of the
manuscript, containing all parts of the paper, must
be submitted on high-density disk. The editor
reserves the right to make corrections, both literary
and technical, to the papers. Papers received are
supposed to have been submitted exclusively to the
Journal of Pakistan Association of Dermatologists
and all authors must give a signed consent to
publication in a letter sent with the manuscript.
Authorship implies a significant contribution. In
case of clinical trials, the names of pharmaceutical
sponsors should be mentioned.
Types of articles
JPAD welcomes original and review articles, case
reports, quizzes, items of correspondence etc.
addressing any aspect of dermatology.
The original article should be of about 2000 words,
with no more than 6 tables or illustrations. Letters
should not normally exceed 400 words and have
more than 10 references.
Parts of the paper
The manuscript should be prepared as below.
Title: In addition to the full title of the paper, a short
version not more than 50 characters, for a running
head, be provided.
Author(s) details: Name(s) of the author(s) should
be given as initial(s) followed by surnames, and
should be clearly linked to the respective addresses
by the use of symbols e.g. , †,‡ etc.
Abstract: All articles other than correspondence
should have an abstract. The original articles should
have a structured abstract comprising of 4
subheadings: background, methods, results and
conclusions. Keywords  5 should be provided to
aid indexing.
Main text: The main text should appear in the
following sequence: introduction, methods, results,
discussion, acknowledgments, references, tables and
legends for illustrations. Each section should begin
on a new page. Generic names of the drugs should
be used. Full names with abbreviations must be
used given with the first mention, thereafter the
abbreviation will be used. Abbreviations should be
used for unwieldy names or where the names occur
frequently. For all quantitative measurements the
International System of Units (SI) should be used.
References
Only papers closely relevant to the author’s work
should be referred to. References should be in the
Vancouver style i.e. references should be written as
unbracketed superscript numbers in the order in
which they appear in the text e.g. ‘our previous
reports1 and that of Cohen et al.2…..’. At the end of
the article, references should give the name(s) and
initials of author(s). If there are more than four
authors, include the first three authors followed by
et al., title of paper, title of the journal abbreviated
in the standard manner (as published in the Index
Medicus), year of publication, volume number, and
first and final numbers of the article, e.g. Grattan C,
Powell S, Humphreys F. Management and
diagnostic guidelines for urticaria and angiooedema. Br J Dermatol 2001; 144: 708-14.
References to books should give the name(s)
followed by initials of author(s) or editor(s), chapter
(if relevant), book title, edition, place, publisher,
year, and pages referred to e.g. Friedman WF, Child
JS. Congenital heart disease in the adult. In: Fauci
AS, Braunwald E, Isselbacher KJ et al., editors.
Harrison’s principles of internal medicine. 14th edn.
New York: McGraw-Hill; 1998. p. 1300-9.
Tables
There should be as few tables as possible and these
should include only essential data. These should be
printed on separate sheets and should be given
Arabic numbers. No horizontal or vertical rules
should be used. Avoid wordy, over-full tables.
Legends should be provided.
Illustrations
Three sets of illustrations should be sent with each
manuscript. Illustrations should be referred to in the
text as ‘Figures’ and be given Arabic numbers.
Each figure should be marked on the back with the
name of the author(s), the title of the paper and the
reference number used in the text. Orientation of the
illustration should be indicated by marking the top
with arrow. Photographs should be unmarked glossy
prints. Diagrams should be on separate sheets and a
legend should be provided for each illustration.
Proofs
Page proofs will be sent, without the original
manuscript, to the corresponding author for proof
correction and should be returned to the editor
within three days. Major alterations from the text
cannot be accepted. Any alterations should be
marked, preferably in red.
221
161