Metabolic & Nutritional Disorders
in Dermatology
By:
Dr. Kazhan Ali Tofiq Kadir
April 2014
The following is a summary of
Metabolic and Nutritional Disorders
most commonly encountered in
practice which manifest themselves
by changes in the skin
hyperlipidaemia
Skin manifestations of hyperlipidaemia include:
flat yellow deposits around the eye
(xanthelasma). Elsewhere on the body they
present as yellowish papules or nodules called
xanthoma. Sudden eruptions can appear in large
numbers over the buttocks, trunk and limbs, or
a few larger lesions can develop on the elbows,
knees, hands and over the Achilles tendon.
Skin Manifestation in DM
Diabetic Dermopathy:
Asymptomatic, resolve spontaneously leaving a scar
usually following improved blood glucose control.
Usually occurs in older diabetic
patients who have
had diabetes >10 years.
Occurs more frequently in
diabetic patients with
retinopathy, neuropathy,
and nephropathy.
Can be indicator of poor
control of blood glucose levels.
Necrobiosis Lipoidica Diabeticorum
Degenerative disease of collagen in the
dermis and subcutaneous fat with an
atrophic epidermis.
Precedes onset of diabetes
in 15-20% of patients
Lesions progress to ulcers
if predisposed to trauma
Location:
85% anterior aspect-pretibial region of lower
extremeties, 15% hands, forearms, face, scalp
Diabetic Bullae
Blisters occur spontaneously in diabetic patients,
atraumatic/asymptomatic lesions on feet and legs.
Patients tend to have adequate circulation in the affected
extremities and peripheral neuropathy.
Three types of Diabetic Bullae:
-Most common: Sterile
containing that heal
fluid
without
scarring.
-Hemorrhagic, heals with
scarring.
-Multiple nonscarring on
sun exposed/tan
skin.
Amyloidosis
Amyloidosis appears in middle age with bruising,
petechiae and purpura related to deposition of
amyloid in the dermal blood supply. This is
most frequently seen in the anogenital,
periorbital and peri-umbilical regions, at the
side of the neck and in the axillae. Atrophic
waxy lesions with areas of purpura inside them
may sometimes be seen, or as shiny smooth
firm flat topped papules of waxy colour.
History:
usually are asymptomatic.
single or multiple lesions.
Physical:
Firm nodules can present anywhere on the skin,
including the face, scalp, extremities, trunk, and
genitalia.
vary from a few millimeters to a few centimeters.
pink to brown or red. waxy, purpuric, yellowish, or
bullous
Lesions tend not to ulcerate.
Macroglossia, a typical feature of systemic amyloidosis.
TREATMENT
Medical Care: topical and intralesional Cs,
cryotherapy, dermabrasion, shaving, curettage
and electrodesiccation, CO2 laser, and PDL.
Surgical Care:
excision and curettage and electrodesiccation
have provided satisfactory cosmetic results.
None of these treatment methods totally
eradicates lesions, which can recur.
The tips of the fingers may exhibit
softening and loosening of the skin
Cutaneous amyloidosis is associated
with various autoimmune/immune
disorders and associations with
sarcoidosis and IgA nephropathy
have been reported.
Background: acanthosis nigricans (AN)
There is association between AN and insulin resistance,
obesity & malignancy
Pathophysiology:
is caused by factors that stimulate epidermal
keratinocyte and dermal fibroblast proliferation.
In the benign AN, the factor is probably insulin or an
insulinlike growth factor that incites the epidermal
cell propagation.
In malignant AN, the stimulating factor is
hypothesized to be a substance secreted either by
the tumor or in response to the tumor.
Exogenous medications also have been implicated as
etiologic factors.
ACANTHOSIS NIGRICANS :
PAPILLOMATOSIS, CONFLUENT & RETICULATED
intertriginous velvety hyperigmentation and discrete and
confluent reticulated brown minimally scaly papules and
patches
This 15-year-old girl complained of dark spots on her chest, abdomen,
and back and velvety darkening of her skin creases for over a year.
Porphyria
There are a number of different forms of
porphyria, e.g. porphyria cutanea tarda,
erythropoietic porphyria…
All are characterized by photosensitivity,
with fragility and blistering of the skin
when exposed to sunlight or ultraviolet
rays.
Porphyrias
Porphyria Cutanea Tarda
Porphyria Erythropoietic
Protoporphyria Erythropoietic
Porphyria Variegate
are a group of inherited or acquired disorders of certain
enzymes in the heme biosynthetic pathway (also called porphyrin
pathway).
They are broadly classified as hepatic porphyrias or erythropoietic
porphyrias, based on the site of the overproduction and mainly
accumulation of the porphyrins (or their chemical
precursors).
accumulation of the porphyrins manifest with either
1- Skin problems or with
2- Neurological complications
(or occasionally both).
Physical: PCT
The most common presenting sign of PCT is fragility of
sun-exposed skin after mechanical trauma, leading to
erosions and bullae, typically on hands and forearms and
occasionally on face or feet.
Healing of crusted erosions and blisters leaves milia,
hyperpigmented patches, and hypopigmented
atrophic scars.
Hypertrichosis is often observed over temporal and
malar facial areas and may also involve arms and legs.
Pigmentary changes include melasmalike
hyperpigmentation of the face.
An erythema or plethora of the central face, neck,
upper chest, and shoulders may be present.
PORPHYRIA CUTANEA TARDA
2-4 mm vesicles and crusts
This middle age man developed increasing skin fragility, blistering
and crusting in sun exposed areas particularly the tops of the
hands
TREATMENT: Medical Care:
Sunlight avoidance is the main defense for
photosensitivity until clinical remission can be
induced.
Alcohol must be proscribed.
Estrogen use should be discontinued, After
achievement of remission, estrogen therapies
may be cautiously reinstituted; however, the
duration of remissions may be shortened.
Remissions may last from several months to
many years. If symptoms recur, re-treatment
can restore remissions.
Therapeutic phlebotomy reduces iron stores, which
improves heme synthesis disturbed.
The goal of therapy is to reduce serum ferritin levels
to the lower limit of the reference range.
Venesections are scheduled at intervals ranging from a
unit of whole blood removed twice weekly to every
2-3 weeks as tolerated by the patient. Care is given
to not induce anemia.
Phlebotomy is the preferred therapy for individuals
with a heavy iron burden.
In patients in whom phlebotomy is not convenient or is
contraindicated and in those who have relatively mild iron
overload, oral chloroquine phosphate (125-250 mg PO
twice weekly) or hydroxychloroquine sulfate (200-400 mg
PO 2-3 times/wk), is often effective.
Larger doses can cause severe hepatotoxicity. Even low-dose
regimens can occasionally produce hepatic toxicity, and
careful monitoring is indicated. Some clinicians begin with a
single, small test dose.
Chelation with desferrioxamine is an alternative means of
iron mobilization when venesections are not practical.
DISORDERS DUE TO VITAMIN
DEFICIENCY
Vitamin A
Phrynoderma, xerophthalmia,
night blindness,keratomalacia.
Vitamin D
Rickets, osteomalacia
Vitamin E
Haemolytic anaemia, ataxia
Vitamin K
Purpura, haemorrhage,
ecchymosis.
CLINICAL FEATURES of Vit. A def.
IN SKIN
Morphology of the lesion is variable may range
from filiform papule to small conical papule to
large papule with large horny centre ,
Color may similar to surrounding skin or may be
slightly hyper pigmented
Back ground skin in affected area is wrinkled
Hands & feet are not involved.
MANAGEMENT
General management –
improvement of diet.
Antibiotic if there is infection.
Special –
Treatment with vitamin A in deficient
cases.
Treatment with other specific vitamins
according to need.
TREATMENT (CONTD.)
Phrynoderma
Local application of 10-40 % Urea cream &
50,000 units of Vitamin A twice a day orally
can make the lesion disappear in 2-3
months.
DISORDERS DUE TO VITAMIN
DEFICIENCY (Contd.)
Thiamin (Vitamin B1) Beri-beri
Riboflavin
(VitaminB2)
Niacin (Vitamin B3)
Vitamin B6
(Pyridoxine)
Biotin
Oro-ocular-genital
syndrome (glossitis,
stomatitis etc.)
Pellagra.
Polyneuropathy
Dermatitis, alopecia,
paraesthesiae.
What is pellagra
Vitamin deficiency disorder
Caused by lack of niacin (B3)
or tryptophan
Symptoms
High sensitivity to sunlight
Dermatitis, alopecia, oedema
Smooth, beefy red glossitis, skin lesions
Insomnia
Weakness
Mental confusion
Ataxia, paralysis of extremities, peripheral neuritis
Diarrhea
DISORDERS DUE TO VITAMIN
DEFICIENCY (Contd.)
Folate
Anaemia
Vitamin B12 Glossitis, hyperpigmentation.
(Cobalamin)
Vitamin-C
(Ascorbic
acid)
Scurvy (swollen and bleeding
gums, redness and swelling in
recently healed wounds (new
wounds may fail to heal)
easily bruised skin
DISORDERS DUE TO
MINERAL DEFICIENCY
Iron
Anaemia, glossitis,
cheilosis,
koilonychia.
Zinc
Acrodermatitis
enteropathica
ACRODERMATITIS
ENTEROPATHICA
Acrodermatitis enteropathica (AE) is a
rare inherited disorder transmitted as
an autosomal recessive trait, caused by
defective intestinal absorption of Zn,
characterized by a triad of acral dermatitis,
alopecia and diarrhea.
Acrodermatitis Enteropathica
Acrodermatitis Enteropathica
Acrodermatitis Enteropathica
Sharply demarcated, brightly erythematous periorificial
plaque in an infant with acrodermatitis enteropathica.
Frequency: AE
1 in 500,000
Mortality/Morbidity:
AE is lethal, usually within the first few years of
life, if left untreated.
Race: No racial predilection exists.
Sex: No sexual preference exists.
Age:
- in the first few months after birth or
- after cessation of breastfeeding.
TREATMENT of AE
Medical Care & MEDICATION:
greater than 1-2 mg/kg of oral zinc
supplementation per day for life.
Prognosis:
Further progression and even death may occur
if AE is left untreated.
Thank You