English

Abstract

INTRODUCTION: We wish to report a very rare case of hand – schuller – christian disease affecting both the twin male children of 6 years age .The diagnosis was made on the basis of classical clinical features, imaging findings, laboratory and histopathology findings. Hand – schuller – Christian is a very rare disease of unknown cause. It is one of the three sub – types of Langerhans cell histiocytosis formerly called as histiocytosis x. LCH is a rare proliferative disorders of bone marrow–derived antigen-presenting cells of the dendritic cell line, also known as Langerhans cells. This disease is a spectrum of three sub-types including eosinophilic granuloma, hand-schuller-christian disease and letterer-siwe disease. We are presenting a case of 6 year old twin brothers who came with complaints of multiple painless swellings over the scalp. Subsequently they were subjected to various investigations including imaging and pathology and a final diagnosis of Hand-schuller-christian disease was given. Eosinophilic granuloma is limited to bone in patients usually 5–15 years old. Hand-Schuller-Christian disease is characterized by multifocal bone lesions and extraskeletal involvement of the reticuloendothelial system (RES) and pituitary gland, usually seen in children 1–5 years old. In Letterer-Siwe disease, there is disseminated involvement of the RES with a fulminant clinical course in children less than 2 years old. The presence of birbeck granules in the cytoplasm is characterictic. In addition tumor cells specifically express HLA – DR, S – 100 and CD1a .