J Med Assoc Thai 2011; 94 (3):135

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Langerhans’ Cell Histiocytosis (LCH) in Adolescence Presented with Central Diabetes Insipidus: A Case Report
Niramitmahapanya S Mail, Deerochanawong C , Suwanbun T , Serirat O , Utrapichart U , Kalayanarooj S , Kuakpaetoon T

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


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