Langerhans cell histiocytosis is a rare disorder of unknown etiology with proliferation of Langerhans’ cells which mayinfiltrate a single or multiple organs. We report a case of 5-year-old child presented with skin lesions since the age of4 months and fever for 10 days where skin biopsy revealed histiocytosis

Kotrashetti, V

Prajapati, Snehal

Sonawane, Vijay B

English

Atharva Scientific Publications (E-Jounals)

Sonawane et al. Langerhans’ cell histiocytosis
Vol 1 | Issue 2 | Jul - Sep 2014 Indian J Child Health 81
Case Report
Langerhans’ cell histiocytosis (histiocytosis X): A rare and diverse 
group of disorder in children
Vijay B Sonawane, Snehal Prajapati, V Kotrashetti
From Department of Pediatrics, DR. D.Y. Patil Medical College, Navi Mumbai, Maharashtra, India
Correspondence to: Dr. Vijay Baburao Sonawane, Assistant Professor, Department of Pediatrics, DR. D.Y. Patil 
Medical College and Hospital, Navi Mumbai - 410 218, Maharashtra, India. E-mail: vijay_ltm@yahoo.co.in
Received – 05 August 2014 Initial Review – 27 August 2014 Published Online – 11 September 2014
Abstract
Langerhans cell histiocytosis is a rare disorder of unknown etiology with proliferation of Langerhans’ cells which may 
infiltrate a single or multiple organs. We report a case of 5-year-old child presented with skin lesions since the age of 
4 months and fever for 10 days where skin biopsy revealed histiocytosis.
Key words: Histiocytosis, Langerhans cell, Skin biopsy
Histiocytosis is a heterogeneous group of disorders that are characterized by proliferation and activation of mononuclear 
phagocyte system. Langerhans cell histiocytosis 
(LCH) is a disorder of unknown etiology with 
proliferation of Langerhans’ cells which may infiltrate 
a single or multiple organs [1]. This disease is more 
common in infants and children under 15 years with 
incidence of one in 200,000 children [2] and adults 
even rarer in about one in 560,000 [3]. It usually 
sporadic but a familial pattern is known. Childhood 
histiocytosis is a diverse group of disorders that may 
present great difficulties for pediatricians in diagnosis 
and treatment as some patients have no symptoms 
while others have symptoms that are mistaken for 
an injury or other conditions [4]. Here, we report a 
case of LCH, diagnosed on skin biopsy since it is 
rare and unclassified entity with diverse clinical 
manifestations.
CASE REPORT
A 5-year-old male child admitted to hospital with 
skin lesions since age of 4 months and fever for 
10 days. Child had been shown to dermatologist on 
the outpatient department basis and been treated 
but no improvement. There is the history of similar 
skin lesions in elder sibling. Child also had a history 
of repeated hospitalization for lower respiratory 
tract infection. On General examination, child was 
undernourished, vital parameters being normal, 
Grade III malnutrition, pallor with significant cervical 
and axillary lymphadenopathy. Systemic examination 
reveals hepatomegaly, 5 cm below the costal margin, 
soft with liver span of 10 cm and splenomegaly of 
3 cm.
Skin lesions were scaly, symmetric, papular, and 
eczematous involving forehead, trunk, back, axilla, 
and chest. Lesions were dry papules and vesicles with 
crusting and oozing fluid mainly on flexor aspects 
(Figs. 1 and 2). Initial differential diagnosis of atopic 
dermatitis and juvenile xanthogranuloma was made.
Diagnostic workup revealed normal complete 
blood count, liver function tests, and skeletal survey 
as bony abnormalities and multiorgan involvement 
is known, ultrasonography Abdomen was showing 
hepatosplenomegaly. Urine Osmolality was normal 
to rule out diabetis insipidus, but skin biopsy from 
upper back right scapular regions showed splitting of 
dermis and epidermis and epidermis showed necrosis 
and parakeratosis while upper dermis showed scanty 
aggregates of oval cells some with indented nucleus. 
Histological features showed inflammatory infiltrate 
Sonawane et al. Langerhans’ cell histiocytosis
Vol 1 | Issue 2 | Jul - Sep 2014 Indian J Child Health 82
of Langerhans cells with multiple eosinophils and 
neutrophils suggestive of LCH (Fig. 3).
DISCUSSION
The childhood histiocytosis constitutes a diverse 
group of disorders which, although individually 
rare, may be severe in their clinical expression. 
Males are more commonly affected than females [5]. 
It is currently thought to be a clonal proliferative 
disorder with highly variable biologic behavior and 
clinical severity [5,6]. The course of the disease is 
unpredictable, varying from rapid progression and 
death to repeated recurrence and recrudescence with 
chronic sequelae, to spontaneous regression and 
resolution [7].
Patients with disease that is localized (i.e., either 
skin or bone) have a good prognosis and is felt to need 
minimum or even no treatment. In contrast, multiple 
organ involvement, particularly in young children 
(<2 years old), carries a relatively poor prognosis [4]. 
The hallmark of LCH is the presence of Birbeck 
granules on electron microscopy [8] and CD1 a 
positivity [4]. But in our patient, immunohistochemistry 
could not be done due to nonaffordability. The 
disease is self-limited in some, whereas in some 
others it is extensive with multi-organ dysfunction. 
About 80% have skeleton involvement, 50% have 
skin involvement, 33% have lymphadenopathy, and 
20% have hepatosplenomegaly [5]. Lymph node: 
Enlargement of the liver in 20%, spleen in 30% and 
lymph nodes in 50% of histiocytosis cases [9]. In 
the present case, patient had no skeletal involvement 
but skin lesions along with lymphadenopathy and 
hepatosplenomegaly. Furthermore, our patient 
improved after 2 weeks indicating self-limiting 
course.
Patients with single system disease can generally 
be cured with conservative management and overall 
survival is about 100% in almost all series. Multi-
system disease without organ dysfunction has been 
associated with, a chronic course, a high rate of 
morbidity and low mortality. Those patients with 
organ dysfunction require polychemotherapy because 
they have a life-threatening disorder [10-13].
Figure 1: Scaly, symmetric, papular, and 
eczematous skin lesions on forehead and shoulder
Figure 2: Skin lesions on axilla, chest, and forearm
Figure 3: Histological features of Langerhans cell 
histiocytosis showing inflammatory infiltration of 
Langerhans cells with eosinophils and neutrphils
Sonawane et al. Langerhans’ cell histiocytosis
Vol 1 | Issue 2 | Jul - Sep 2014 Indian J Child Health 83
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Funding: None; Conflict of Interest: None Stated 
How to cite this article: Sonawane VB, Prajapati S, 
Kotrashetti V. Langerhans’ cell histiocytosis (histiocytosis 
X): A rare and diverse group of disorder in children. 
Indian J Child Health. 2014;1(2):81-3.


Langerhans’ cell histiocytosis (histiocytosis X): A rare and diverse group of disorder in children

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Langerhans’ cell histiocytosis (histiocytosis X): A rare and diverse group of disorder in children

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