A Case of Xanthoma Disseminatum with Diabetes Insipidus / 대한피부과학회지

ABSTRACT

Xanthoma disseminatum (XD) is a rare, potentially progressive non-Langerhans cell histiocytosis, which preferentially affects males in their childhood or young adulthood. It is characterized by the insidious onset of small, yellowish-red to brown papules and nodules that are discrete and disseminated. They characteristically involve the eyelids and flexural areas of the axillary and inguinal folds, and the antecubital and popliteal fossae. Systemic disease frequently occurs, the most common manifestation being diabetes insipidus (DI), which occurs in about 40% of cases and is due to deposition of xanthoma cells in the hypothalamic-pituitary axis. We present a case of XD with DI, which was treated with the combination therapy of CO2 laser vaporization, cyclophosphamide, electrocauterization and synthetic anti-diuretic hormone.