Xanthoma disseminatum: case report.

Xanthoma disseminatum is a rare, benign, non-Langerhans' cell histiocytic disorder of unknown etiology. A case is presented of a 71-year-old man with a three-year history of disseminated symmetric yellowish papules and plaques on the skin of the face, neck, flexor regions, trunk, extremities and oral mucosa, with fatty infiltration of the liver and pancreas, and cardiac complaints. Xanthomatous rhinophyma predominated on the face. Clinical, immunohistochemistry and histology findings indicated the diagnosis of xanthoma disseminatum. Although the patient had a positive family history of cardiovascular diseases and a number of symptoms that are often associated with hyperlipidemia, repeat plasma levels were always within the normal limits. The case was interesting because of a number of etiologic factors that could be connected with the appearance of xanthomas in our patient. Therefore it was difficult to classify the disease into one of well-defined nosologic entities. The case report is supplemented with a review of relevant literature.