Langerhans’ cell histiocytosis with twenty-nail dystrophy and a rare cutaneous manifestation

Abstract

Langerhans’ Cell Histiocytosis (LCH) constitutes a rare group of idiopathic disorders, characterized by proliferation and infiltration of various tissues by clonal population of cells with phenotype of langerhans’ cell. We report a 2 year old female child, a product of consanguinous marriage who presented with seborrhoeic dermatitis like eruption involving scalp, twenty nail dystrophy, oral lesions and loosened teeth, mild hepatomegaly, low RBC count, multiple osteolytic lesions in skull and pelvic bones thus falling into the category of high-risk multisystem disease. Following the clinical suspicion of LCH in the child, a battery of cost-effective investigative tools were employed primarily to confirm the diagnosis and to decide the extent of systemic involvement. This case is being reported from the view point of its rarity and the diagnostic challenges posed to some dermatologists before the child was brought to our department.

KEYWORDS: Histiocytes, Langerhans, Dermatologists