Histiocytosis-X in gynecology: a case presentation and review of the literature.

A patient is presented with persistent vulvar ulcerations of 15 months' duration. Biopsies of the lesions were diagnostic of histiocytosis-X. Chest x-ray revealed a honeycombed reticulonodular pattern consistent with pulmonary histiocytosis-X. The dehydration test indicated diabetes insipidus. Electron microscopy revealed Langerhan granules characteristic of histiocytes. This patient represents one of approximately two dozen cases in the world literature showing gynecologic manifestations of histiocytosis-X. The presentations, diagnosis, therapy, and prognosis of this disease are discussed.