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134]

Review Article

Leprosy in Children

Abstract Tarun Narang,

Children are believed to be the most vulnerable group to infection with Mycobacterium leprae due Bhushan Kumar1
to their immature or nascent immunity and exposure to intrafamilial contacts. The child proportion Department of Dermatology,
among newly detected cases of leprosy is a strong indicator of continued transmission of the Venereology and
disease and one of the main targets of the current World Health Organization strategy is  “to reduce Leprology, PGIMER,
transmission of the disease and reduction of grade‑2 disability among new child cases.” Despite an Chandigarh, 1Department
effective treatment and global achievement of leprosy elimination, the childhood leprosy proportion of Dermatology, Shalby
has not improved significantly. Leprosy in children does not just affect a child’s health like other Multispeciality Hospital,
diseases; they can be stigmatized, bullied, and shunned for their lives. Hence, effective planning to Mohali, Punjab, India
bring down the incidence of leprosy and its complications in children should become a top priority.
Regular school surveys and annual contact surveys for early detection of cases is therefore an
important tool in achieving the goal of elimination of leprosy. In addition to continuing to administer
multidrug therapy to patients, new preventive approaches need to be considered to break the chain of
transmission and reach zero disease status.

Keywords: Chemoprophylaxis, childhood, disability, immunoprophylaxis, leprosy

Introduction sensory loss or muscle weakness. Hence,

Leprosy is a chronic mildly infectious
disease caused by Mycobacterium leprae.
It predominantly effects the skin, mucosa,
and nerves. It has been a major public
health problem in many developing
countries despite the availability of an
effective treatment. Leprosy was universally
“eliminated as a public health problem”
18 years ago, yet new cases continue to be
seen in endemic areas and 9 out of every
100 new cases diagnosed today are children.
The current scenario was aptly described by
Dr Erwin Cooreman, Team Leader of the
World Health Organizations (WHO’s) Global
Leprosy Programme. “The world has the
tools, the right medicines and the political
will  –  yet we are falling short of detecting
the disease in time, particularly among
children.”[1] Children are believed to be the
most vulnerable group to infection with
M. leprae due to their immature or nascent
immunity and exposure to intrafamilial
contacts.[1] Leprosy is a great masquerador
and may present as an ill‑defined
hypopigmented asymptomatic patch on face
or arms to diffuse infiltration of the entire
skin and neuromuscular symptoms such as
in areas where leprosy is still prevalent, it
should figure in differential diagnoses, not
only among the dermatologists but also
physicians/neurologists/pediatricians who
are involved in medical care of children and
adolescents. In the absence of an effective
vaccine, early diagnosis and treatment is
essential in the prevention of disabilities
and deformities, and reduce the physical,
psychosocial, and economic burden of the
disease.[2]
Epidemiology
Global scenario
The introduction of the WHO multidrug
therapy (MDT) has played a pivotal role
in achieving the epidemiological target of
“elimination of leprosy as a public health
Address for correspondence:
problem.” Elimination was attained at a Dr. Bhushan Kumar,
global level in 2000, whereas India achieved Shalby Multispeciality Hospital,
the same in December 2005. Although the Mohali, Punjab, India.
WHO MDT has been our main weapon E‑mail: kumarbhushan@
hotmail.com
in our fight against leprosy, with more
than 16 million treated leprosy cases and
a current world prevalence of only 0.25; Access this article online
the annual new case detection rate and the
child rate have not decreased significantly Website: www.ijpd.in

suggesting unabated active transmission of DOI: 10.4103/ijpd.IJPD_108_18

the disease. Reports from 150 countries of Quick Response Code:

This is an open access journal, and articles are
distributed under the terms of the Creative Commons WHO’s six regions show that of the total
Attribution‑NonCommercial‑ShareAlike 4.0 License, which
allows others to remix, tweak, and build upon the work
non‑commercially, as long as appropriate credit is given and the
new creations are licensed under the identical terms. How to cite this article: Narang T, Kumar B.
Leprosy in children. Indian J Paediatr Dermatol
For reprints contact: reprints@medknow.com 2019;20:12-24.

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Narang and Kumar: Childhood/ pediatric leprosy
of 210,671 newly diagnosed cases of leprosy during 2017,
16,979  were children, representing almost 7.5% of all new
cases reported annually.[3]
The child proportion among newly detected cases of
leprosy is a strong indicator of continued transmission of
the disease and a significant pool of undiagnosed cases in
the community. Globally, this proportion among total cases
has shown a considerable variation within different regions,
for example, in Africa, the childhood proportion has ranged
from as high as 38.25% in Comoros to as low as 1.12% in
Burundi.[3]
Indian scenario
According to a recent report by the National Leprosy
Elimination Programme, from a total of approximately
135,485 new cases of leprosy in India, 8.7% were among
children.[4]
The proportion of child cases was more than 10% of
new cases detected in 10 States/UTs, namely Tamil Nadu
(17.64%), Maharashtra  (10.18%), Bihar  (13.70%), Dadar
and Nagar Haveli  (19.79%), Arunachal Pradesh  (10.71%),
Punjab (17.25%), Nagaland  (11.75%), Daman and Diu
(14.29%), and Lakshadweep (11.11%).
The demographic characteristics of the children affected by
leprosy have been evaluated in many studies in India either
by school surveys or hospital‑based studies with contact
assessment. A substantial variation in the prevalence rates
of childhood leprosy from 4% to 34% has been observed
in these studies conducted in different parts of the Indian
subcontinent.[2,5‑9] However, this statistical disparity in
prevalence could be due to variation in the cutoff upper
age limit for childhood case definition  (ranging from
<14 to <19 years) in different studies [Table 1].
The priorities and targets of leprosy control programs
are constantly changing with the changing epidemiology
of leprosy. One of the main targets of the current WHO
strategy is “to reduce transmission of the disease and
reduction of grade‑2 disability (G2D) among new child
cases.”[20] The current G2D rate in children is high, and it
might be higher than what the statistics reveal as a number
of cases are not reported.[1]
Age and sex distribution
Leprosy is known to occur at all ages ranging from early
infancy to old age. Among children, the disease tends
to occur with the highest frequency in 5–14  years of
age group and up to 6% cases are below 5  years of age.
Higher frequency in older children may be due to the long
incubation period of leprosy (5–7 years), delay in diagnosis
of early lesions and difficulty in assessing the sensory loss
in younger children.[2,15] The youngest patient diagnosed
to have leprosy was only 3 weeks old, from Martinique, a
small island near West Indies.[21] Brubaker et  al. reported
91 infants under 1 year of age diagnosed with leprosy
Indian Journal of Paediatric Dermatology | Volume 20 | Issue 1 | January-March 2019
thereby disapproving a mistaken belief that leprosy is
exceedingly rare or nonexistent in the very young.[22]
Among children, boys are more commonly affected than
girls;[13,23] however, this may not represent the true statistics
as detection in girls may possibly be lower than boys due
to neglect of the female child and greater mobility and
increased opportunities for contact in the male child.
Transmission and possible sources of infection in
children
One of the most important sources of infection in
childhood cases is familial contact with leprosy. The risk of
developing leprosy in a person is four times when there is a
neighborhood contact. However, this risk increases to nine
times when the contact is intrafamilial. Further, the risk
gets higher (up to 14 times) if the contact has MB form
especially lepromatous disease[13] and when the index case
is mother[23] or where the number of patients was more than
one. Detection of a case of childhood leprosy may provide
an opportunity to detect the index case, usually within the
family and sometimes even community. The prevalence of
familial contact in childhood leprosy has ranged from 10%
to 36% as observed in different studies [Table 1].[5‑9,15,23‑26]
The exact mode of transmission of leprosy is still not
conclusively proven although infection by nasal droplets
and direct contact with skin are believed to be the main
routes. Other factors that may influence transmission
include genetic susceptibility, extent of exposure, and the
environmental conditions. Epidemiological evidence of
transplacental transmission of leprosy is also present.[27] The
report of a child developing leprosy at the age of 3 weeks
is an example where the infection could have been
intrauterine. Although M. leprae are known to be present
in the breast milk of mothers suffering from lepromatous
leprosy (LL), the risk of acquiring leprosy infection in the
breastfed infant through the gastrointestinal tract remains
uncertain.[24]
Transmission by skin to skin contact between baby and the
mother is more likely as compared to ingestion through
breast milk.[28] Inoculation leprosy occurs by transmission
of M. leprae through the broken skin after trauma is also a
distinct possibility in children.
There is evidence indicating possible nonhuman sources
such as soil and water, insect vectors and the free‑living
amoebae (Acanthamoeba spp.) may be the reason for
unabated transmission of leprosy.[29‑31] Early studies had
shown that a large number of bacilli are discharged
through washing of mouth, nose‑blows, and from skin
by lepromatous patients[32‑34] and these bacilli are known
to remain viable in the environment for 9 days or even
longer.[35] The location of lesions on the exposed sites
in children suggests the role of contact with surfaces
contaminated with M. leprae. However, most of these
studies are polymerase chain reaction (PCR)‑based
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Narang and Kumar: Childhood/ pediatric leprosy

studies and although M. leprae may remain viable in the

BT (46.9)
et al.[19]

(0‑14)
environment for a variable period it may not replicate and

10‑14

0.014
Balai

2.2:1
28.1

68.7

12.5
2.3

7.2
34
‑

‑
infect the people exposed to it hence virulence of these
organisms has to be proved by animal studies.[31]
et al.[18]

(0‑19)
13‑18
Pathogenesis
Gitte

44.1

17.6
17.4
11.2

PB
16

68
‑

‑
Leprosy is a chronic granulomatous disorder caused by
M leprae. Leprosy is mildly infectious which is evident

MB (95.5)
et al.[17]
Ramos

2.6:1

36.4

27.3

38.9
by the fact that the majority of the people  (about 90%) do
7.4
‑

‑
‑

‑
not develop clinical manifestations of leprosy due to their
innate immune response or defense mechanism against
Sasidharanpillai

M leprae. The immunologic response to M. leprae consists

6‑12 (0‑14)

PB (73.18)
BT (65.9)

of two components: innate and acquired immunity. Innate

et al.[16]
12.1

immunity is determined by genetic factors, including alleles

‑
‑

‑
‑

‑
‑

‑
‑
of the PARK2/PACRG gene[36] as well as other genes.[37]
In a genome‑wide association study of patients in China,
Table 1: Clinico‑epidemiological studies on pediatric leprosy from India

variants of genes in the nucleotide‑binding oligomerization

MB (52.5)
BT (67.8)

Multiple

domain‑2‑mediated signaling pathway (which regulates the

et al.[9]
Dogra

(0‑18)

(57.6)
11‑14

3.9:1
4.81

25.4

28.8
81.4

33.9
40.7

89.8
5.1 innate immune response) were found to be associated with
susceptibility to leprosy.[37] The cells of monocyte/dendritic
cell origin are important mediators in immune‑pathogenesis
12.8 (grade
PB (81.2) MB (51.7)
BT (78.7) BT (66.3) BT (70.8) BT (68.75) BT (70.3)

of leprosy.[38] Individuals with sufficient exposure and

et al.[15]

2 only)
Singal

Single
(0‑14)

(52.9)
11‑14

2.3:1
14.5

19.8

18.6

80.2
1.16
9.6

70

susceptibility to M. leprae may develop a broad range of

clinical manifestations, depending on the host’s ability to
mount an acquired immune response to infection. This
Multiple

cellular immune response appears to be controlled by a

Rao[14]

(0‑18)

(62.5)
11‑15
11.43

2.5:1

6.24
3.12
18

25
‑

‑
‑

number of nonhuman leukocyte antigen genes such as

tumor necrosis factor‑alpha, interleukin (IL)‑10, vitamin D
BT ‑ Borderline tuberculoid; PB ‑ Paucibacillary; MB ‑ Multibacillary; SSS ‑ Slit‑skin smear

receptor, and PARK2.[39] Although it is difficult to quantify

24 (grade
PB (71)
Grover
et al.[13]

2 only)
(0‑14)

the relative contribution, the genetic factors are important

1.4:1
7.06

21.9

22.6

14.6
<14

‑
‑

risk factors which control not only the susceptibility

to leprosy; they also play an important role in the
development of different phenotype of leprosy, reactions,
Multiple

PB (83)
et al.[12]

(0‑14)

(63.4)
11‑14
Jain

38.8

60.8

29.7
3:2
9.8

9.4

and prognosis.[40]
‑

‑
‑

The clinical manifestations of leprosy depend on the

interplay between innate and acquired immune responses
Sehgal and Kumar
Chaudhry[11] et al.[10]

(0‑14)
1.9:1
0‑14
4.5

involving interactions of the bacterial proteins with immune

‑

‑
‑
‑
‑
‑

‑
‑

components of the host. These interactions may either

prevent the invasion and infection or promote their growth
Single (59.6)
10‑14 (0‑19) 8‑14 (0‑14)

PB (78.3)

and development of leprosy. The immune system has evolved

2.6:1
5.06

4.34
BT
8.7

3.1
18

primarily to combat infection, but in leprosy, the immune

‑

‑
‑

response is responsible for the broad clinical spectrum of

the disease and similar to an autoimmune disease, seems to
trigger further complications such as nerve damage.[41]
Multiple
et al. [10]

BT (59)
Kumar

(54.5)
2.2:1
12.4

19.7

17.4
66.6

7.4
6.8
PB

Nutrition and leprosy

‑
‑

Leprosy has long been known as a disease of poverty as

most of the countries where leprosy is still endemic are
Treatment completion (%)
Child proportion rate (%)
Commonest age group,

underdeveloped or developing and leprosy itself along

Nerve thickening (%)
Number of lesions in
Commonest clinical

with its disabilities and stigma further pushes them toward

SSS positivity (%)
Sex (male:female)

Classification (%)

poverty. Poverty is considered an important risk factor for

Deformities (%)
Reactions (%)
spectrum (%)
years (range)

leprosy susceptibility, although nutritional deficiencies

majority (%)

Relapse (%)
Contact (%)

may be the major contributing factor yet the mechanisms

underlying this association and other factors still need to be
identified. Some studies have shown a positive association

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Narang and Kumar: Childhood/ pediatric leprosy
between food shortage or food insecurity and leprosy, and it
was suggested that impaired host immune response is a result
of malnutrition. Improving dietary diversity or nutrition of
people living in high‑prevalence communities can be tried as
one of the measures to control the transmission of leprosy.[42]
Possible outcomes of leprosy infection in children
The course of childhood leprosy is unpredictable.
Progression or regression of lesions is common. There may
be three possible outcomes when a child is infected with
M. leprae; (a) the child does not develop leprosy; (b) early
lesions like indeterminate leprosy may appear, which may
remain stationary or disappear due to self‑healing; and
(c) the indeterminate lesion/s may progress to a determinate
type. In the absence of a specific marker for susceptibility
and immunological status, it is difficult at present to predict
the outcomes in contacts.[43]
The phenomenon of self‑healing has been reported in
earlier studies; however, the observation period was not
long enough to establish its permanence, but Lara and
Nolasco observed a cohort of sanitarium‑born children who
showed self‑healing lesions for almost 25 years. In about
75% of these children, the healing was sustained even
throughout stressful events in their adult life, implying that
self‑healing leprosy in children is frequent and sustained.[44]
Classification
A five group clinico‑histological classification based on
immunological status described by Ridley and Jopling
is still the most widely accepted.[45] The revised Indian
classification by IAL in 1981 divided leprosy into
five broad groups, namely indeterminate, borderline,
tuberculoid, lepromatous, and polyneuritic. A  simplified
classification based on the number of lesions was given by
WHO in 1998; up to 5 lesions  (paucibacillary  [PB]) and 6
or more lesions/with anyone nerve thickened (MB).[46]
Leprosy in children presents predominantly as PB
disease.[47] The spectrum of leprosy in children is reported
mostly to be tuberculoid (TT), borderline tuberculoid (BT),
mid‑borderline (BB), and indeterminate forms. Single skin
lesion and indeterminate leprosy were seen to be a common
early presentation in most of the studies. BT leprosy is the
Table 2: Important differential diagnoses of leprosy in children
Hypopigmented patches* Erythematous/skin coloured plaques*
Pityriasis alba Sarcoidosis
Pityriasis versicolor Lupus vulgaris
Vitiligo Histiocytosis
Morphoea Mycoses fungoides
PKDL PKDL
Nevus depigmentosus
*In all these conditions, lesional sensations are well preserved, which is a very helpful sign in older children. However testing of sensations
is difficult in small children. PKDL ‑ Postkala‑azar dermal leishmaniasis
Indian Journal of Paediatric Dermatology | Volume 20 | Issue 1 | January-March 2019
most common clinical type with the prevalence ranging
from 42% to 78% of all childhood cases in different studies
[Table 1].[5,7‑9,13,15,23,24] LL can be seen in advanced cases but
seems to be rare during the first few years of life. Histoid
leprosy and pure neuritic leprosy are rare in children.
Clinical presentation and disease spectrum in childhood
leprosy
The data on clinical presentation and disease spectrum in
childhood leprosy are mostly derived from hospital‑based
studies [Table 1]. The most common presentation in these
studies is a single hypopigmented patch predominantly
on the exposed body parts with normal or impaired
sensations.[5,7‑9,13,15,23,24,47] However, few studies have also
reported low rate of single skin lesions.[10,12,14] The clinical
presentation in the form of bilateral and symmetrical
ill‑defined macular hypoesthetic lesions, diffuse infiltration
of the face and earlobes, are less commonly observed
because of the rarity of BB, BL, and LL disease in
children. Neuromuscular symptoms like trophic ulcers
due to sensory loss or muscle weakness are occasionally
the first to be observed. Higher rates of thickened nerves
(60%–80%) have also been observed in some of the
studies.[9,10‑12] Rarely children with LL may present with a
history of chronic nasal discharge instead of epistaxis due
to chronic rhinitis and nasal congestion. Table 2 gives a
list of common differential diagnosis in childhood cases of
leprosy.
Indeterminate leprosy
Indeterminate leprosy (IL) is an early and transitory stage
of leprosy characterized by one or more hypopigmented
macules. Indeterminate leprosy may evolve over a period of
2–5  years and may spontaneously resolve or establish into
one of the subtypes in the spectrum of leprosy. Towards the
end of indeterminate stage, there may be signs of subtle
neurological deficits such as decreased sweating and/or a
loss of thermosensitivity, whereas pain sensitivity is still
intact. It usually presents with macular lesions with fairly
well‑defined edges and slight or no sensory loss, usually
over the covered areas of the body [Figure 1]. This
clinical ambiguity in the diagnosis of IL [Table 2] is
further compounded by negative slit skin smears (SSS)
Sensory or motor weakness
Inherited peripheral nerve disorders e.g., hereditary sensory and
sensory‑motor neuropathies of various types
neurofibromatosis
neuropathies associated with developmental defects
acquired neurological conditions like iatrogenic, and other
injection injuries to the sciatic nerve and traumatic neuropathies
15
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Narang and Kumar: Childhood/ pediatric leprosy
for acid‑fast bacillus  (AFB) and the nonspecific histology
which reveals chronic inflammation with the predominance
of lymphohistiocytic infiltrate in the perineural, intraneural,
and periadnexal areas. Molecular diagnostic tests like
multiplex PCR can help in the diagnosis of IL with limited
clinical manifestations.[48] However, one has to be very
careful while giving a diagnostic label of leprosy in a child
for obvious psychological trauma and social stigma.
Nodular leprosy of childhood
Nodular leprosy (NL) of childhood is a benign clinical
variant of tuberculoid leprosy. The lesions are usually
localized on cheeks, limbs, and buttocks [Figure 2] and
may result from the intimate skin contact with parents or
relatives who have LL. On histopathology, NL lesions are
characterized by dense granulomas with a greater number
of confluent tubercles in comparison to the classical
tuberculoid lesions. Strong stimulation of cell‑mediated
immunity against M. leprae evoked by the inoculation of
bacilli into the skin has been thought to be responsible for
the stability and auto‑resolution of NL of childhood.[49]
Tuberculoid (TT), borderline, and lepromatous leprosy
TT disease and LL disease represent the two ends of the
leprosy spectrum which is associated with good and poor
immunity against M. leprae, respectively. Between these
two ends lies the unstable group designated as borderline
leprosy.[50]
Good immunity: Tuberculoid or paucibacillary
leprosy (TT)
Polar tuberculoid form of leprosy is characterized by
solitary papules and plaques which may coalesce into
sharply demarcated erythematous or skin colored plaques
with raised borders and an annular appearance in an
asymmetrical distribution. Neurological examination reveals
anesthesia or decrease in thermal, touch, and pain sensitivity
as well as anhidrosis usually in the center of the lesion. The
lesions of TT can undergo spontaneous resolution.
Poor immunity: Lepromatous leprosy
The LL form initially appears as hypochromic, diffuse, and
symmetric lesions which eventually involve the entire skin
and the skin appears erythematous or reddish brown, shiny
and later on infiltrated with papules or nodules  [Figure 3].
The characteristic features of LL are diffuse infiltration of
the face, loss of the lateral third of the eyebrow (madarosis);
and infiltration of the earlobes [Figure 4]. The sensory loss
or NFI is symmetrical and observed late in MB spectrum
as compared to the PB spectrum; the temperature, pain and
touch sensitivity is lost in that order. It is a systemic disease
involving internal organs, such as the eyes, larynx, pharynx,
liver, spleen, suprarenal glands, kidneys, bone marrow,
and lymph nodes due to the lack of cellular immune
response or specific anergy to M. leprae. SSS is strongly
positive with live, regular staining AFBs [Figure 5], and
16 Indian Journal of Paediatric Dermatology | Volume 20 | Issue 1 | January-March 2019
the histopathology reveals diffuse macrophage infiltration
in the dermis; cytoplasm of many of these macrophages
is loaded with bacilli or globi with large quantities of
lipids, which on staining give the appearance of foam
cells (Virchow cells). The infiltrate is separated from the
epidermis by collagenous fiber bands  (Unna band) giving
the appearance of subepidermal clear zone.[2]
Intermediate stage: Borderline leprosy or dimorphic
leprosy
Borderline leprosy is an immunologically unstable
form, as it presents clinical, bacteriological, and
histopathological characteristics, which can shift either
toward the TT pole (borderline tuberculoid; BT),
or the LL spectrum (borderline‑borderline, BB or
borderline‑lepromatous, BL).[10]
Lesions in BL often begin as multiple, small, nearly
symmetrically distributed hypopigmented to coppery‑hued
macules that have indistinct borders that merge into the
intervening normal skin. Sometimes, these lesions coalesce
to form bigger patches which tend to be distributed
symmetrically. A decrease in appendages and sweating,
which are features of the disease in the BT and TT
spectrums, are not prominently seen in BL. With time
as the disease downgrades toward lepromatous pole, the
macules become infiltrated beginning from the center
and forming plaques and nodules [Figure 6]. BB is the
most immunologically unstable portion of the borderline
spectrum. Patients quickly move either toward the
lepromatous or the tuberculoid poles. Lesions characteristic
of both the tuberculoid and lepromatous types, are seen in
this part of the spectrum. Skin lesions, which are in the
form of infiltrated papules, plaques, and even nodules at
times, tend to be symmetric. The characteristic lesion of
BB leprosy is an annular plaque with a well‑demarcated
“punched‑out” inner margin and a sloping outer margin,
giving a “swiss cheese” appearance [Figure 7]. Lesions in
BT often resemble those in TT, but are more numerous,
larger in size, and less well defined. Unlike TT, where the
outer margin is well defined, lesions in BT have an outer
margin that at places slopes toward the surrounding skin.
BT lesions are characterized by “finger‑like” extensions,
known as pseudopodia [Figure 8]. There may be small
satellite lesions surrounding the plaque. The lesions
are often dry, scaly, hypoesthetic plaques with loss of
appendages, and decreased sweating; but not as marked
as seen in lesions of TT. The nerves are asymmetrically
and irregularly thickened in BT leprosy. The patient may
present with localized anesthesia and motor deficits.[50]
Musculoskeletal manifestations, such as arthralgia, arthritis,
and myalgia, are known to occur as a part of reactions,
however, chronic polyarthritis, asymmetric polyarthritis
with predominantly rheumatoid distribution can occur in
the absence of reaction due to direct synovial infiltration
by M. leprae. It is erosive, deforming, and responds
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Narang and Kumar: Childhood/ pediatric leprosy

Figure 1: Indeterminate leprosy. Solitary hypopigmented, ill-defined lesion

on the face of a child
Figure 2: Nodular leprosy of childhood. Nodular lesion on the cheeks of
a girl

Figure 4: Lepromatous leprosy; diffuse infiltration of the ears

Figure 3: Diffuse infiltration of skin in a case of lepromatous leprosy

Figure 6: Multiple erythematous infiltrated plaques on the face in a child

Figure 5: Slit skin smear showing multiple uniformly staining, slender bacilli with BL disease
in a case of lepromatous leprosy

Figure 7: Mid borderline leprosy, annular plaque with a well-demarcated

“punched-out” inner margin and a sloping outer margin, giving a “swiss Figure 8: Well defined plaque of borderline tuberculoid leprosy with
cheese” appearance pseudopodia and satellite lesion

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Narang and Kumar: Childhood/ pediatric leprosy
poorly to anti‑leprosy treatment. Neder et  al. and Chopra
reported musculoskeletal manifestations such as asymmetric
polyarthritis of the joints of the hands as a manifestation
in 14% of the examined children.[2,51,52] Aberrant
immunological response in leprosy leads to the production
of numerous autoantibodies such as RA, ANA, and ANCA
and this may create diagnostic dilemma in patient of
chronic polyarthritis, raising the possibility of co‑occurrence
of juvenile rheumatoid arthritis, and lupus erythematosus.
However, anti‑cyclic citrullinated peptide (anti‑CCP) is less
likely to be found positive in leprosy arthritis cases.
Reactions
Leprosy reactions are acute/subacute inflammatory
processes, mediated by T lymphocytes or by antibodies,
which are present before, during, and after treatment. The
triggering or precipitating factors can be MDT, infections,
parasite infestations, vaccinations, physical or emotional
stress and should always be addressed while managing
the reactions. Lepra reactions are subdivided into type 1
reactions, which results from spontaneous enhancement
of cellular immunity and delayed‑type hypersensitivity
to M. leprae antigens, and type 2 reactions or erythema
nodosum leprosum, mediated by immune complexes and
proinflammatory cytokines. In childhood leprosy, reactional
episodes and disabilities are less frequently seen. The
frequency of reactions in children is reported to vary
from 3.1% to 33.9%[5,7‑9,13,15,23,24] as compared to adults
where more than 50% of the patients develop reactions.
Older children and children with borderline disease
are at higher risk for reactions.[2,53] Higher incidence of
reactional episodes, both type 1 and 2 occurring with
or without neuritis in children has also been observed,
mostly in hospital‑based studies.[9,12] Severe reactions
are accompanied by fever, malaise, anorexia, swelling
of the face and extremities, and neuritis. Rarely, the only
manifestation of a reaction can be isolated neuritis, which
presents as spontaneous pain, nerve tenderness, and NFI.
Lepra reactions are the main causes of neural damage and
deformities and should be identified and treated urgently as
well as adequately to prevent disabilities.[54]
Deformities and disability
Children with irreversible deformity and disability face
many difficulties in education, social life, and day‑to‑day
activities, which differ to some extent from their adult
counterparts. The prevalence of deformities associated
with childhood leprosy in India varies from 0.5% to
40.7%  [Table 1]. One of the targets of the Global Leprosy
Strategy is Zero new child cases with G2D. According
to the WHO weekly epidemiological report 2017, this
indicator was reported by 120 countries, of which 88
reported 0  cases, and 27 reported, 1–10 and five countries
reported more cases.[3] Majority of these deformities
involve upper limbs.[9] Various factors responsible for
increased risk of deformities in childhood leprosy have
18 Indian Journal of Paediatric Dermatology | Volume 20 | Issue 1 | January-March 2019
been identified. The presence of neuritis at presentation
or later on, older children, MB disease (smear positivity,
multiple skin lesions, and multiple nerve involvement)
significantly increases the risk of deformities.[55] It was also
observed that children with thickened nerve trunks are at
6.1 times higher risk of developing deformities compared
to those who did not have nerve enlargement.[55] Other
factors contributing to the development of deformities
include; delay in accessing health care and lepra reaction
at the time of presentation. Children with these risk factors
should be followed up more diligently so as to detect the
onset of any deformity and initiate management as early as
is possible.[14]
Monitoring for new nerve function impairment
The assessment of NFI like sensory loss or motor
weaknesses and nerve thickening should be done at the first
visit to stratify the subsequent risk of developing new NFI
and based on the risk category the frequency of monitoring
can be determined in patients [Table 3] to ensure that
any new primary impairment will be detected early. With
early detection, there is a greater chance of reversal with
treatment, before the onset of secondary impairments as
MDT is not likely to reverse the NFI. Monitoring might
include home testing, by the parent or the child, and formal
nerve function assessment with SW filaments, thermal
testing, and motor function assessment by the treating
physician. Since there is little evidence on the outcomes of
new NFI in children, deciding whether to prescribe steroids
in a particular situation entails balancing individual risks
and benefits.[56]
Diagnosis
Accurate diagnosis is important in children but it can be
very difficult at times. For instance, single hypopigmented
patch on the face in children has high risk of misdiagnosis,
since there are numerous common causes of hypopigmented
patches [Table 2] in this age group. Elicitation of sensory
impairment in younger children may be quite difficult on
the facial lesions as well as otherwise. Moreover, the fact
that indeterminate leprosy may progress to more definitive
forms of leprosy,[57] emphasizes the need for greater
caution and diligence in early diagnosis and early initiation
of treatment among children. Since the demonstration
of classical histological features of leprosy or AFB in
cutaneous lesions of IL is difficult; hence, a reasonable
period of observation is recommended if the diagnosis
cannot be confirmed at the time of presentation.
Slit‑skin Smear examination
Slit‑skin smears, from the suspected lesions of leprosy,
should be stained for acid‑fast bacilli (AFB) to estimate the
number of M. leprae when present, (bacillary index, BI) and
the proportion of viable bacilli (Morphological Index, MI).
Smears are positive in LL, BL, and sometimes BT cases.
However, a large proportion of early cases of childhood
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Narang and Kumar: Childhood/ pediatric leprosy
Table 3: Clinical prediction rule for probability of new nerve function impairment: Risk of new nerve function
impairment during the first 2 years after diagnosis
Nerve function assessment
No nerve function impairment at diagnosis
With nerve function impairment at diagnosis
Risk category
Low risk
Moderate risk
High risk
NFA ‑ Nerve function assessment; PB ‑ Paucibacillary; MB ‑ Multibacillary
leprosy is AFB negative because most of them are TT, BT,
or indeterminate. Positive skin smears have been reported
in  <10% cases in many previous studies. Some of the
recent studies have reported higher smear positivity rates
ranging from 17.4% to 30%  [Table 1]. The skin smear
positivity has been shown to increase with age.[55]
Histopathology
Histopathological examination of skin biopsy is
considered better than SSS and some experts believe that
histopathology with Fite staining if done properly can be
more useful than SSS. Granuloma formation may not be
observed in children due to immature immune system.[47]
The clinicopathological correlation in childhood leprosy
has ranged from 37% to 93% in various studies.[8,14]
Newer methods  (serology and molecular diagnostic
methods)
None of the currently available tests like SSS, skin biopsy
is sensitive enough so the current diagnostic challenge is to
identify or develop a point‑of‑care test that can contribute
to or facilitate early diagnosis and classification of leprosy.
Serological tests for antibodies against M. leprae antigens
such as phenolic glycolipid‑I (PGL‑I), or conjugated
antigens leprosy anti–natural octyl disaccharides- Leprosy
Infectious Disease Research Institute diagnostic‑1
or  NDO‑LID  can assist in the diagnosis and classification
of leprosy. Furthermore, the assessment of these antibodies
may also help to monitor the effectiveness of treatment,
and help in prognosis and even prediction of reactions.[58]
A major challenge for leprosy control and elimination is
to identify individuals who will develop leprosy after
exposure and to take steps to prevent illness especially
among the contacts of leprosy patients.[59] People with
positive anti‑PGL‑1 antibody have 2.7 times higher odds
of developing overt disease and it has shown promise in
predicting the development of leprosy among contacts and
school children; however, it might not be useful in the
detection of PB cases.[59,60] PGL‑1 has been widely studied,
but its diagnostic potential as a single marker in India
may be limited due to cross‑reactivity with Leishmania
donovani.[61] Serological markers cannot be used as
confirmatory tests for diagnosis of leprosy, however, they
can be used as a supplementary test in endemic settings
Indian Journal of Paediatric Dermatology | Volume 20 | Issue 1 | January-March 2019
PB cases MB cases
1% (low risk) 16% (moderate risk)
16% (moderate risk) 67% (high risk)
Frequency of NFA
Educate about reporting promptly if any deficit is noticed, then
re‑assessed every 6 months for 2 years
NFA every three months, for at least 24 months
NFA monthly for 12 months, then quarterly for up to 24 months
while planning for chemo or immunoprophylaxis to the
contacts or general population.[59,60]
M. leprae specific genetic markers such as the 16s rRNA,
or 16S rDNA, RLEP, and TTC repetitive sequences have
the potential to diagnose early leprosy in childhood, with
sensitivities of approximately 80% and 30% in MB and PB
cases, respectively.[62,63]
Treatment
MDT is the mainstay of leprosy treatment. The WHO
recommended fixed duration multidrug treatment for
6 months in PB leprosy and 12 months in cases of MB
leprosy.[64] Parental education is essential before starting
MDT for the child. Parents and young patients need to
understand the following [Box 1].
The dosage regimen, of both PB and MB MDT for children,
according to the different age groups, has been shown
in Tables 4 and 5. In children <10 years of age the doses
should be preferentially calculated according to the weight
of the child: dapsone 2 mg/kg/day, rifampicin 10 mg/kg,
and clofazimine 1 mg/kg/day daily and 6 mg/kg monthly.
Fortunately, tolerance to standard antileprosy drugs is good
in children. Available MDT blister packs though convenient
are not child‑friendly. Treatment dropout rates in children
range from 10% to 20% in some programs,[15,65] main cause
being the child’s refusal to cooperate in swallowing tablets.
Child‑friendly treatment options such as flavored syrups are a
need of the hour for improvement in dosing and compliance.
Alternative regimens
Children with dapsone hypersensitivity syndrome are usually
offered a modified WHO MDT regimen consisting of rifampin
monthly and clofazimine daily at the usual doses and same
duration. In children with “single skin lesion leprosy” (smear
negative) without any nerve involvement, the single dose
rifampin, ofloxacin, and minocycline combination  (ROM)
has been used. However, ROM is no longer supplied through
the WHO, and most national programs do not endorse it.
Minocycline is generally contraindicated in early childhood.
Quinolones, such as ofloxacin have been shown to cause
arthropathy (degenerative changes in weight‑bearing joints)
in young animals, so have to be used with caution in children
and adolescents. However, field trial using single‑dose
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Narang and Kumar: Childhood/ pediatric leprosy

regimen of minocycline and ofloxacin in children for the
treatment of single lesion PB leprosy did not show significant
adverse effects. Any child with rifampicin resistance
requires an individually tailored regimen, based on the
recommendations from the WHO expert committee,[66] with
due regard to the risks that all second‑line drugs carry for
children.[67] There is not enough data regarding the safety of
newer antileprosy drugs in children.
Response and follow‑up
The erythema and induration of skin lesions may diminish
within a few months of initiating therapy. It may take a few
years for cutaneous lesions to resolve fully, depending on
the initial number of lesions and severity of infection. Most
lesions heal without scarring. Once killed, dead bacilli are
removed from the tissues very slowly; some may persist in
the tissues for several years hence mere smear positivity
should not mean nonresponsiveness or drug resistance, it
is important to establish viable bacilli by mouse footpad or
RNA probes. The NFI does not improve significantly with
treatment and patients should be taught to evaluate these
Box 1: Education for parents of children to be started on
multidrug therapy
MDT rapidly renders a case noninfectious, so there is no need for
segregation
Isolating the child and his/her things is unnecessary; normal
domestic hygiene suffices
How to recognize the common and serious adverse effects of MDT
and what to do in case of any adverse event?
Do not expect a rapid disappearance of skin lesions after starting
treatment
MDT is available for free from all government hospitals
Need for safe storage of the medication at home
Leprosy reactions may occur despite taking MDT correctly. Do not
stop MDT and consult the doctor for treatment of reaction
How to recognize leprosy reactions and what to do?
MDT ‑ Multidrug therapy
Table 4: Dosage of multidrug therapy for children with
paucibacillary leprosy
PB‑MDT
Age group Rifampicin: Monthly Dapsone: Daily dose,
(years) dose, supervised (mg) unsupervised (mg)
10‑14 450 50
15 or above 600 100
Duration: 6 months. PB ‑ Paucibacillary; MDT ‑ Multidrug therapy
areas on hands and feet regularly for evidence of injury so
as to obtain treatment promptly. Special protective shoes
with soft insoles made of microcellular rubber may be
needed to avoid injury or ulceration. Motor loss resulting
in deformities may require corrective surgery.
Treatment of neuritis and reactions
Children with reactions (both type 1 and 2) require the
prompt use of steroids for the prevention of nerve damage
and deformity. Clofazimine can also be used for management
of both type 1 and 2 reactions, it is generally recommended
at 1.5–2  mg/kg three times daily for 1  month, then
reducing by one dose per day each month. The maximum
dose of 300 mg daily can be administered. Other drugs
which can be used for reactions are hydroxychloroquine,
methotrexate, azathioprine, cyclosporine, and nonsteroidal
anti‑inflammatory drugs like paracetamol, etc. Thalidomide
is not recommended for children below 12 years old, due
to the lack of safety information, but it might be used  –  if
legally available  –  in adolescent boys. Rehabilitative
measures such as physiotherapy and corrective surgeries
should also be offered to selected patients.
Preventive measures
Preventive measures for leprosy include early diagnosis and
management of active cases as well as contact management.
Household contacts should be evaluated annually for
evidence of disease for at least 5 years and should be
educated to seek immediate attention if suspicious skin or
neurologic changes develop. Hence, community education
about leprosy along with mandatory household contact and
school surveys if implemented nationally would result in
the reduction of disease burden.[68]
Vaccines in leprosy
The ideal leprosy vaccine should induce strong,
long‑lasting T‑cell responses directed against M. leprae,
thereby preventing disease, limiting infection, and
furthermore, reducing bacterial transmission.[68] Several
vaccine strategies have used whole mycobacteria in the
immunoprophylaxis; however, to date, none besides Bacille
Calmette–Guérin  (BCG) and Mycobacterium indicus
pranii  (MIP) vaccines have advanced into common use.
There is an emerging need in leprosy research to further
evaluate the available vaccines such as BCG or MIP for
leprosy prevention and immunomodulation and develop
new subunit or recombinant vaccines. Newer vaccines like
a LepVax (a tetravalent vaccine) has been tested in mice,

Table 5: Dosage of multidrug therapy for children with multibacillary leprosy

MB‑MDT
Age group Rifampicin monthly Dapsone daily Clofazimine
(years) supervised dose (mg) unsupervised dose (mg) Unsupervised dose (mg) Monthly supervised dose (mg)
10‑14 450 50 50 every other day 150
15 or above 600 100 50 daily 300
Duration: 12 months. MB ‑ Multibacillary; MDT ‑ Multidrug therapy

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Narang and Kumar: Childhood/ pediatric leprosy
wherein it was found to reduce bacillary load and delay
motor NFI and may prove useful in humans.[69]
BCG remains the only vaccine widely administered for
the prevention of leprosy and has played an important role
in leprosy control. Systematic meta‑analyses indicate that
BCG has a protective efficacy of around 50%, and that
protection appears to be better against the MB than PB
forms. The protection rates with BCG revaccination have
varied between 26% and 61%. The level of protection
varies greatly with an overall protection of 41% in trial
studies and 60% for cohort studies. Moreover, the efficacy
was significantly more for household contacts  (66%) when
compared to the general population.[70] Although the effect
diminished with age, no variation was observed in the
BCG action in relation to the age on application of the
first dose of the vaccine, the revaccination increased its
response. Merle et  al. found no additional protective effect
when applying revaccination with BCG, but they did report
that a longer follow‑up time may well reveal the benefits
of the second dose.[71] However, the WHO guidelines do
not support BCG revaccination, and there is a concern that
it may also accelerate the onset of PB leprosy patients.[71]
BCG has also been used in combination with killed M.
leprae and M. vaccae, but this did not significantly increase
the protective efficacy.
In an attempt to make BCG more immunogenic and
to extend its protective lifespan, several investigators
have genetically refined the bacteria. Recombinant BCG
strains that over‑express antigens that are homologous
with M. leprae like Ag85 complex, or rBCG that secretes
M. leprae major membrane protein‑II (also known as
bacterioferritin; ML2038) have shown better efficacy in
inhibiting the multiplication of bacilli in mouse foot pad
as compared to BCG. However, these are BCG vaccines
might not work in populations that have been successfully
primed with current BCG strains.
In the south Indian vaccine trial comparing BCG,
BCG + killed M. leprae, Mycobacterium w  (M.  w) (now
called MIP) and ICRC vaccines, ICRC provided the best
protection, at 65.5%. However, the widespread use of
ICRC for the prevention of leprosy has not been reported.
MIP, previously M.  w, has also been investigated as an
immunoprophylactic measure against M. leprae.[72] In
the south Indian vaccine trial, MIP provided only 25.7%
protection. The protective efficacy was better in the Uttar
Pradesh M. w trial where protective efficacies of 69%, 59%,
and 39% were reported after 3, 6, and 9 years, respectively,
when only the contacts were vaccinated, and the efficacy
was 68%, 60%, and 28% when both patients and contacts
were vaccinated[72] and the vaccine efficacy was highest in
children. The waning of immunity after 9 years suggests
the utility of booster dose. MIP has also been found to
be effective as a immunotherapeutic modality in few
studies from India. It was seen that the MIP vaccine led
Indian Journal of Paediatric Dermatology | Volume 20 | Issue 1 | January-March 2019
to a faster reduction in bacillary load along with decrease
in the frequency and severity of type 2 reactions without
exacerbating type 1 reactions and neuritis. It can be used as
an adjunct to MDT in leprosy patients with a high bacillary
load.[73]
Chemoprophylaxis in high‑risk contacts
Leprosy contacts are at highest risk of acquiring leprosy
hence some researchers have suggested chemoprophylaxis
in high‑risk contacts, that is, contacts with MB disease
and seropositive to anti‑PGL‑I cases, using single‑dose
rifampicin (SDR), 600 mg for adults above 35 kg of body
weight, 450 mg for children above 9 years of age, and adults
20–35  kg, and 300  mg for children weighing  <20  kg.[74]
The protection rate for chemoprophylaxis varies from 30%
to 70% and even a minimal reduction in incidence among
the contacts can significantly affect the incidence in
countries like India, recommending its use in public health
policies.[75] SDR treatment is being practiced by the Indian
National Leprosy Programme since November 2017.[76]
Experts believe that SDR may be a cheap and easier
intervention, but it does not prevent MB leprosy hence it
is unlikely to have an effect on transmission. It may also
not help contacts of MB patients or contacts with high
anti‑PGL‑I levels as their bacillary load may be too high to be
eliminated by a single dose. It may also not help individuals
who acquire infection after the dose as drugs do not provide
immunological memory as opposed to vaccines. Moreover,
it may lead to the development of rifampicin‑resistant M.
leprae, and that will be a big blow to the leprosy control
program in endemic countries like India.[77]
Conclusion
Leprosy in children is a strong indicator of recent
transmission of the disease and failure of leprosy control
programs. Leprosy elimination as a public health problem
has been misunderstood as “zero leprosy.” The skill for
diagnosing and managing leprosy is diminishing leading
to missed and misdiagnosis of leprosy in infants and
young children. This delay in diagnosis transforms into
deformities and disabilities which have a significant
bearing on the physical, psychosocial, and financial aspects
on children and their families. Fear of social ostracism may
dissuade children or their families from seeking medical
care at an early stage. Children have a low frequency
of voluntary reporting for leprosy care, unless there are
serious consequences. Hence, effective planning to bring
down the incidence of leprosy and its complications in
children should become a top priority. Regular school
surveys and annual contact surveys for early detection
of cases is, therefore, an important tool in achieving the
goal of elimination of leprosy. In addition to continuing
to administer MDT to patients, new preventive approaches
need to be considered to break the chain of transmission
and reach zero disease status.
21
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Narang and Kumar: Childhood/ pediatric leprosy
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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24 Indian Journal of Paediatric Dermatology | Volume 20 | Issue 1 | January-March 2019