Endocrine Abstracts

Langerhans cell (LCH) histiocytosis occurs in 1 per 560,000 adults with variable manifestations. It has a high rate of misdiagnosis due to its variable presentation and rarity. It is even more rare that it presents with simultaneous and multiple endocrine dysfunction early in the course of disease. We report a case of Langhans Cell Histiocytosis in an adult male presenting with central Diabetes Insipidus, Hypergonadotrophic Hypogonadism and pulmonary disease. A 36 year old male presented with new onset polyuria, polydipsia, nocturia associated with weight loss and generalised lethargy. Systemic enquiry revealed exertional dyspneoa, dry cough, two episodes of spontaneous epistaxis and left leg pain resulting limp on walking. Chest examination revealed bilateral crackles in lung bases. Investigations showed compensated hypergonadotrophic hypogonadism, marginally elevated prolactin, normal thyroid function and IGF 1. Short Synacthene Test was normal. Water Deprivation Test suggested Central Diabetes Insipidus. His ESR was elevated at 51. Vasculitis screen, immunoglobulin and compliment were normal. Pituitary MRI revealed absent bright spot. Chest X ray showed nodularity on upper lobes and CT chest confirmed upper lobar nodularity, cystic changes and fibrosis. Skeletal survey was normal. Diagnosis was confirmed by lung biopsy. A diagnosis of multisystem Langhans Cell Histiocytosis with Central DI and Hypogonadism was made. He was started on desmopressin and testosterone replacement. Langhans Cell Histiocytosis should be considered as a diagnosis in any case presenting with Cental Diabetes Insipidus with multiorgan involvement.