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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

Najeeb Shah, Harshal Deshmukh, Muhammad Jawaid Akbar, Shah Rukh Malik, Shahbaz Nazir, Somil Rastogi, Shiva Mongolu

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.