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ISSN 2007-5294 (Print)
Confederación Nacional de Pediatría de México, A. C. (CONAPEME)

2013, Number 1

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Pediatr Mex 2013; 15 (1)

Histiocytosis of Langerhans cells

Meza PJA, Puig RBA, Uribe JE, Fajardo D

Language: Spanish
References: 17
Page: 22-25
PDF size: 76.39 Kb.


ABSTRACT

Histiocytosis is a group of proliferative disorders, reactive or neoplasic of the phagocytic mononuclear and dendritic cell system. It often occurs in children as a cutaneous disease. The cutaneous Langerhans Cell Histiocytosis is rare and heterogeneous in its clinical presentations. Its diagnosis results in a clinical challenge because of its ability to mimic other entities. The course of the disease is characterized by either spontaneous resolution or multivisceral dissemination with poor prognosis. We report a case of a 5-month-old female infant suffering from cutaneous Langerhans’ Cell Histiocytosis as a sole manifestation. Extensive investigation failed to reveal any other signs of the disease in other organs. The cutaneous lesions were restricted to the scalp, face, fingers, toes, and perineum, as well as the external genitalia. These consisted of erythematous desquamative skin lesions, along with pustules, papules and some areas of necrotic scarring lesions. The diagnosis was based on histopathologic examination of skin biopsies revealing a histiocytic infiltrate positively staining with protein S100 antibodies and CD1a. Oral treatment with prednisone and TMP/SMX was administered. Under this regimen, within a 2-month period a satisfactory resolution was observed without any emergence of further skin manifestations.


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