Langerhans cell histiocytosis (LCH) is a rare neoplastic lesion characterized by accumulation of cells having major phenotypic features of skin Langerhans cells. It usually affects young children but rare cases were also reported in adults. The clinical spectrum of LCH includes Letterer–Siwe disease, Hand–Schüller–Christian disease, eosinophilic granuloma, Langerhans cell sarcoma as well as int...

Diseases characterized by proliferative changes in the reticulo-endothelial system, other than the leukaemias and specific infections such as tuberculosis and typhoid fever, give rise to three main syndromes in children, but there are many atypical and intermediate forms. Originally described as three separate conditions, these syndromes, namely Letterer-Siwe disease, Hand-Schiiller-Christian s...

A case of Langerhans Cell Histiocytosis (LCH) is reported in 1½ years old boy. He had seborrhoeic dermatitis like condition of scalp, papular lesions with purpura typical of Letterer-Siwe disease associated with constitutional symptoms, hepatosplenomegaly, jaundice, anaemia and thrombocytopenia. Peripheral blood film and bone marrow examination showed presence of LCH cells.

Eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease are three disorders characterized by proliferating histiocytes. Collectively, they are called histiocytosis X. Histiocytosis X often presents with lytic calvarial lesions [1]. Hypothalamic involvement is the most common intracranial manifestation [2]. Solitary histiocytosis X within the cerebral hemispheres, alth...