Adult-onset Langerhans cell histiocytosis presenting with adipsic diabetes insipidus, diabetes mellitus and hypopituitarism: A case report and review of literature

摘要

Langerhans cell (LCH) histiocytosis occurs in 1 per 560,000 adults with variable manifestations. However, it rarely presents with simultaneous and multiple endocrine dysfunctions early in the course of the disease. We report a case of LCH in an adult manifesting with adipsic diabetes insipidus, diabetes mellitus and hypopituitarism.A 41-year-old Filipino male presented with polyuria and polydipsia. He was initially diagnosed as a simple case of diabetes mellitus but symptoms did not fully resolve after control of glucose. He had loss of thirst perception, erectile dysfunction and memory impairment. These were followed by crusting skin lesions over the face, trunk and extremities. He was hospitalized because of altered mentation because of severe dehydration, hypernatremia and hyperglycemia. Subsequent work-up showed thickened hypothalamus and infundibulum on magnetic resonance imaging. Skin biopsy and immunostaining with S-100 and CD1a confirmed the diagnosis. There was partial improvement after chemotherapy. He was maintained on desmopressin and testosterone.Multiple endocrinopathies can be the presenting signs of adult-onset LCH. They can be harbinger of subsequent involvement of other organs. Central diabetes insipidus, other hypothalamic dysfunctions and anterior pituitary hormonal deficiencies, when present at the onset as in this case, will likely have poor response to treatment despite improvement of other non-endocrine organs. The permanent lesions in the hypothalamus and pituitary gland warrants continued endocrine evaluation and intervention.