Langerhans Cell Histiocytosis Presenting with Central Diabetes Insipidus in an Adult: A Case Report with a Discussion on Differential Diagnosis, Diagnostic Work up and Treatment

Langerhans Cell Histiocytosis (LCH) is rare in adults; the condition affects 1-2 patients per million annually in the general population. Diabetes Insipidus (DI) is a common manifestation of Langerhans Cell Histiocytosis (LCH) although its reported frequency varies in different series. The pathogenesis of the condition is not precisely understood. Infiltration of the hypothalamic-pituitary axis by Langerhans-like cells has been reported in 50% of autopsied patients. Central Diabetes Insipidus (CDI) can be familial, idiopathic or secondary and is a disorder characterised by polyuria, polydipsia and formation of hypotonic urine.