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Childhood Leprosy A Review

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Review Article
Childhood Leprosy: A Review
Abstract
Leprosy in children is of special importance as it is the indicator of transmission in community.
It affects both the child and family members psychologically and functionally. In this review, we
will discuss regarding epidemiology of childhood leprosy in detail, types of leprosy in children,
diagnostic difficulties in children, prevention of disabilities in children, and effect of childhood
leprosy on the community.
Keywords: Childhood leprosy, community transmission, deformities, diagnosis, treatment
Introduction
Leprosy is a disease known for its
paralyzing deformities and social stigma.
It is sad and unfortunate that even though
WHO has declared leprosy to be eliminated
in 2005, it has remained as endemic disease
in various parts of the world specifically
developing countries like India.[1] Although
leprosy affects all age group, leprosy in
children is of special importance as it is an
indicator of transmission in the community.
In this review, we have discussed regarding
epidemiology of childhood leprosy in detail,
types of leprosy in children, diagnostic
difficulties in children, prevention of
disabilities in children and effect of
childhood leprosy on community.
Epidemiology
A total of 135,485 new cases were detected
during the year 2016–2017, which gives
Annual New Case Detection Rate of
10.17/100,000 population, as against
127,334 cases in 2015–2016. A total of
88,166 leprosy cases are on record as on
April 1, 2017, giving a prevalence rate
of 0.66/10,000 population, as against
86,028 cases in April 1, 2016.[2] A high
proportion (49.57%) of the new leprosy
cases detected during 2016-2017 were
multibacillary that included (8.7%) of
children and grade II deformity was
reported in 3.87%. A total of 11792 child
cases were recorded indicating the child
case rate of 8.7%. Proportion of child
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112
cases was more than 10% of new case
detected in 10 States/Union territories,
namely (i) Arunachal Pradesh-10.71%, (ii)
Bihar - 13.70%, (iii) Jharkhand - 10.59%,
(iv) Maharashtra - 10.18%, (v) Nagaland 11.76%, (vi) Punjab - 17.25%, (vii) Tamil
Nadu - 17.64%, (viii) Dadra and Nagar
Haveli - 19.79%, (ix) Daman and Diu14.29%, and (x) Lakshadweep-11.11%.
Swetalina Pradhan,
Bibhu Prasad
Nayak1,
Gaurav Dash
Department of Dermatology,
All India Institute of Medical
Sciences, Bhubaneswar,
1
Department of Pediatrics,
SCB Medical College, Cuttack,
Odisha, India
Classification of Leprosy
Although various types of classifications
have
been
proposed
for
leprosy,
Ridley‑Jopling classification is the most
accepted classification as it includes clinical,
immunological,
and
histopathological
spectrum of the disease. According to the
above classification leprosy is of five types,
tuberculoid(TT), borderline tuberculoid (BT),
mid-borderline(BB), borderline lepromatous
(BL) and lepromatous(LL).[3] For field
healthcare workers, WHO has classified
leprosy into two broad categories basing
upon number of skin lesions: Paucibacillary
(PB) and Multibacillary (MB). Paucibacillary
(PB) leprosy is defined as five or fewer skin
lesions without detectable bacilli on the
skin. Multibacillary (MB) leprosy defined as
six or more lesions and may be skin smear
positive.[4]
In majority of studies, the age group that
is most commonly affected by the disease
among children under 15 years of age was
found between 10 and 14 years of age,
which can be justified by the disease’s
long incubation period of approximately
3–5 years.[5‑12] However, Santos et al.[13]
identified a discrete predominance in the
Address for correspondence:
Dr. Swetalina Pradhan,
Department of Dermatology,
All India Institute of Medical
Sciences, Bhubaneswar,
Odisha, India.
E‑mail: dr.swetalinapradhan@
gmail.com
Access this article online
Website: www.ijpd.in
DOI: 10.4103/ijpd.IJPD_47_18
Quick Response Code:
How to cite this article: Pradhan S, Nayak BP,
Dash G. Childhood leprosy: A review. Indian J
Paediatr Dermatol 2019;20:112-6.
© 2019 Indian Journal of Paediatric Dermatology | Published by Wolters Kluwer - Medknow
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Pradhan, et al.: Childhood leprosy: A review
5–9‑year‑old age group, in 54.54% (42/77), followed by
10–14‑year‑old age group 44.15%. Although theoretically
leprosy should not occur in infants because of long
incubation period of leprosy in years, it has been reported in
infants as young as 2–3 months of age by Brubaker et al.[14]
59.38% of the cases, with a predominance of ulnar nerve.[9]
Similar findings were reported by Singal et al. with 70%
of the patients having thickening of nerve, out of which
nearly half of the patients had multiple nerve involvement
with ulnar nerve being the most common.[11]
PB leprosy is commonly found in children as compared
to MB because of good immunity than adults.[8] In
contrast, two studies found more number of multi-bacillary
(60.65% and 91.66%) cases in children.[15,16] In various
studies, it has been found that BT leprosy is the most
common form found in children followed by BL leprosy,
indeterminate, lepromatous leprosy, and pure neuritic
leprosy [Figures 1 and 2].[8,15‑17]
Diagnosis of Childhood Leprosy
Lepra Reaction in Children
In general, lepra reaction is rare in children under 15
years of age. Majority of the studies have reported the low
frequency of lepra reaction in children varying between
1.36% and 8.33%.[5,8,10,16,17] However, some studies have
reported greater percentage than expected varying from
18% to 29.7%.[6,7,11,18,19] In all of these studies, the type 1
reaction was most commonly found, which is expected,
given that the most frequent clinical form was BT. Type 1
reaction is associated with neuritis. Severe neuritis can lead
to sudden nerve palsy leading to deformities such as foot
drop, wrist drop, and clawing. Hence, neuritis should be
treated with adequate dose of corticosteroid to bring down
inflammation and damage in the nerves.
Nerve Involvement in Children Affected with
Leprosy
Peripheral nerve involvement is the main cause of
deformities in children affected with leprosy and associated
lepra reaction compounds the problem.[20] Jain et al. found
the involvement of peripheral nerves in 186 patients of
a total of 306 evaluated children, with more than one
thickened nerve in most cases; the ulnar nerve is the most
affected.[6] In 2009, Rao reported polyneural involvement in
Figure 1: Borderline tuberculoid leprosy: Hypopigmented anesthetic patch
covering almost entire face
Diagnosis of leprosy is based on the cardinal signs of
leprosy. The patient should have anaesthetic skin lesions,
enlarged and/or tender peripheral nerve, and slit skin smear
positive for acid-fast bacilli. Children usually present
with PB leprosy where getting acid‑fast bacilli in slit
skin smear not always possible. Hence, diagnosis purely
based on clinical examination most of the times. The most
challenging part for a physician is to elicit sensory loss to
fine touch in children which is impossible and inaccurate.
Although pain sensation can be elicited, the child will not
cooperate for the same and pain sensation is always last to
go among all sensations. One of the easy techniques which
can be done is checking for temperature sensation. One
can take two test tubes containing warm and cold water.
The patient is asked to close eyes and examiner can ask
by touching the test tubes on skin to appreciate whether
cold or warm. In doubtful cases, one can go for biopsy
for confirmation of diagnosis. In a study, 35% of doubtful
cases showed findings of Hansen’s disease in histology.[21,22]
It has been found in various studies that the skin lesions of
leprosy usually undergo self‑healing in children.[23,24] Hence,
in doubtful cases, one can wait and keep the child under
observation (untreated) for a period to see whether the skin
lesion is improving or definite sensory loss is appreciated.
However, in endemic areas and with family members being
affected with leprosy, it is always wise to start treatment.
Treatment of Leprosy in Children
In children with PB leprosy, rifampicin and dapsone are given
for 6 months, and those with MB leprosy and clofazimine is
Figure 2: Borderline lepromatous leprosy: Hypoesthetic hypopigmented
macules over trunk
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Pradhan, et al.: Childhood leprosy: A review
given in addition to rifampicin and dapsone. The dose of the
drugs is different according to the age group [Table 1].
Childhood Disability Due to Leprosy and
Prevention of Disability
Disability is the most common cause of social stigma in
leprosy. Leprosy causes disabilities through damage to
peripheral nerves. Disabilities and deformities frequently
persist even after successful treatment and affect
psychologically and functionally both the children and their
family members. It not only affects day‑to‑day activities in
children but also can affect their education, interaction with
their surrounding though studies pertaining to these aspects in
children are lacking. In various studies, it has been found that
children affected with leprosy have their initial presentation
with deformities which suggests lack of awareness.[7,18] Kar
and Job suggested that children with neural thickening have
6.1 times more chances to develop deformities as compared
to those without neural thickening. In their study, out of 275
children under 15 years of age, 10.5% cases had deformities,
with the following risk factors: late diagnosis, multiple
cutaneous lesions, MB disease, positive slit skin smear,
various affected nerves, and reactional state at the time of
the diagnosis.[7] The same risk factors were also found in the
study by Singal et al. with 12.8% incidence of deformities in
the hands, feet, and eyes.[11] Hence, the most important factor
to prevent disability in leprosy patients is early detection and
adequate treatment of neural impairment.
Effect of Childhood Leprosy on Community
The main transmission and acquisition pathways for leprosy
are the upper airways.
Leprosy in children indicates continuing transmission in
the community.[14] Source of infection in children could
be familial or nonfamilial close contacts.[6] Household
contact increases the risk of transmission to 9 folds in
children because of early and continuous exposure to the
leprosy bacillus.[25] In a study from South India, family
history of leprosy was present in 18·2% of the childhood
cases and all the affected parents and grandparents who
were the household contacts of these children had smear
positive, multibacillary leprosy.[15] Community‑based
survey of leprosy patients in defined rural and urban areas
of Maharashtra state (2007), has found that 19% of the
children in the studied rural area and 47% of the children
in urban area had history of close contacts with leprosy
Age
10‑14 years
patients.[26] Hence houshold contact surveys should be an
effective way of case finding when the index case is a child.
Conversely, children in a household where a new leprosy
case has been diagnosed are at increased risk compared
with the general population and even compared with adults
in the household: the risk to children aged 1–14 years was
much higher than in older people.[11]
Resistance
The emergence of drug resistance is the main cause for
concern in leprosy because limited number of drugs are
available for treatment. Usually, a combination of more
than two drugs, with different mechanisms of action, taken
regularly for a sufficient period, will prevent the emergence
of drug resistance. Resistance to rifampicin, dapsone, and
quinolones is reported due to mutations in the binding sites
of these drugs in large number of samples by molecular
biological methods.[27,28] Clofazimine and minocycline
resistance have not yet been reported. Development of
resistance to first‑line drugs is becoming serious threat to
the efficacy of existing multidrug therapy (MDT) program.
Patients suspected to be rifampicin resistant are also
expected to be resistant to dapsone. In the year 1998 WHO,
technical advisory committee recommended the following
regimen for adults with suspected rifampicin resistance:[29]
1. Daily administration of 50 mg of clofazimine, together
with 400 mg ofloxacin and 100 mg of minocycline for
6 months, followed by
2. Daily administration of 50 mg clofazimine, together
with 100 mg of minocycline or 400 mg of ofloxacin,
for at least an additional 18 months.
Newer drug regimens suggested for leprosy in 2009 by
the “WHO Report of the Global Programme Managers’
Meeting on Leprosy Control Strategy”[30,31]
For rifampicin susceptible MB patients, a fully supervised
monthly regimen could include Rifapentine 900 mg
(or rifampicin 600 mg), moxifloxacin 400 mg, and
clarithromycin 1000 mg (or minocycline 200 mg) for
12 months. For rifampicin‑resistant patients, the intensive
phase could include moxifloxacin 400 mg, clofazimine
50 mg, clarithromycin 500 mg, and minocycline 100 mg
daily supervised for 6 months. The continuation phase could
comprise moxifloxacin 400 mg, clarithromycin 1000 mg,
and minocycline 200 mg once monthly, supervised for an
additional 18 months.
Table 1: WHO multidrug therapy regimen for children
PB
MB
Day‑1 (once a month)
Day‑1 (once a month)
Rifampicin 450 mg and Dapsone 50 mg
Rifampicin 450 mg, clofazimine 150 mg and dapsone 50 mg
Day 2‑28
Day 2‑28
Dapsone 50 mg daily
Clofazimine 50 mg alternate day and dapsone 50 mg daily
Full course‑6 blister packs
Full course‑12 blister packs
For children younger than 10, the dose must be adjusted according to body weight. PB ‑ Paucibacillary; MB ‑ Multibacillary
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Pradhan, et al.: Childhood leprosy: A review
A single‑dose combination of rifapentine, moxifloxacin,
and minocycline killed 99.9% of the viable Mycobacterium
leprae and was more bactericidal than a single dose of
rifampicin, ofloxacin, and minocycline or rifampicin alone.
In the same study, it was also observed that the combination
of moxifloxacin‑minocycline was more bactericidal than
the combination of ofloxacin‑minocycline.[32,33]
These drugs such as ofloxacin, moxifloxacin (quinolone),
and minocycline are contradicted in childrens. No alternate
regimens are designed for children.
Drug Reactions
In cases of severe adverse reactions, an alternative
multidrug therapy regimen is recommended.[34] In adults,
the alternative regimens use ofloxacin (quinolone) and
minocycline (tetracycline), which are contraindicated in
children under 10 years of age, due to the risk of the
early closure of the epiphysis as well as dental and bone
alterations, respectively.
Relapse
Mostly relapse occurs in leprosy due to inadequate
treatment. It is an important quality of service indicator in
assessing the long term efficacy of MDT. The predisposing
factors include the presence of persister bacilli,
monotherapy (dapsone), inadequate or irregular therapy, the
presence of multiple skin lesions and/or thickened nerves,
etc. The majority of relapse cases in a study belonged to
the 12–15 years age group. Nearly 80% of the relapses
occurred during PB treatment, and 70% had received
previous treatment from other institutions. Inadequate
treatment of MB as PB might have contributed to higher
relapsing rate.[35]
In another study,[5] despite completing 6 months of MDT‑PB
prescribed according to body weight, relapses occurred in
13 out of 784 children with PB leprosy. Three occurred
during the 2‑year surveillance period after completion of
treatment, and another five, two, and three occurred 6, 1 2,
and 1 8 months after surveillance.
Counseling
Parent must be counseled about the duration of treatment
and to complete treatment without default. In addition, they
should be informed regarding the signs and symptoms of
lepra reactions and to report to the physicians immediately
while encountered with such sign and symptoms. In
children having anaesthesia of hands and feet, the parents
should be taught how to take care of anaesthetic hands
and feet to avoid trophic ulcers. Parents and siblings of the
index case must be examined for leprosy.
Conclusion
In summary, the incidence of childhood leprosy still high in
various states of India. Diagnosis of leprosy in children is
difficult compared to adults. In case of doubt, it is better to
keep the child under observation for few months, however,
in endemic areas, it is always wise to treat the cases at the
earliest. The parents should be warned regarding the signs
of both types of lepra reactions, so that the treatment can
be instituted to avoid deformities due to nerve damage
in type 1 reaction and systemic complications in type 2
reactions. All the family members should be examined for
evidence of leprosy and treated.
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form the patient(s) has/have
given his/her/their consent for his/her/their images and
other clinical information to be reported in the journal. The
patients understand that their names and initials will not
be published and due efforts will be made to conceal their
identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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