Langerhans’ Cell Histiocytosis (LCH) in Adolescence Presented with Central Diabetes Insipidus: A Case Report
Sathit Niramitmahapanya MD*, Chaicharn Deerochanawong MD*, Tawatchai Suwanbun MD*, Onsiri Serirat MD*, Usakorn Utrapichart MD**, Siripan Kalayanarooj MD**,Thiti Kuakpaetoon MD**
Affiliation : * Department of Medicine, Rajavithi Hospital, College of Medicine, Rangsit University, Bangkok, Thailand ** Department of Radiology, Rajavithi Hospital, College of Medicine, Rangsit University, Bangkok, Thailand
A 19-year-old Thai man presented with symptoms of central diabetes insipidus, exopthalmos and multiple ostolytic lesions of the cranial vault. Skin biopsy showed strongly positive CD 1a and S100. Electron microscopy showed rod shape and pentalaminar granules called “Birbeck” granules, a diagnosis compatible with Langerhans’ cell histiocytosis (LCHs) or histiocytosis X. He was successfully treated with systemic chemotherapy.
Keywords : Langerhans’ cell histiocytosis (LCHs), Histiocytosis X, Diabetes insipidus, Birbeck
