Xanthoma Disseminatum

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Abstract
• A 55-year-old man with the classical mucocutaneous lesions of xanthoma disseminatum has been followed up for a period of 13 years. The special features of this case, which make it unique, are as follows: (1) the availability of histologic data on multiple lesions for more than a ten-year period; (2) the progressive nature of the multiple osseous lesions; (3) the metabolic studies that show no evidence for accumulation of abnormal sterols in a xanthoma, the blood, or intestinal aspirate; (4) the development of hypothyroidism and symptoms or signs, or both, of an intracerebral and an intraspinal lesion; (5) the partial regression of the cutaneous symptoms and lesions while receiving clofibrate, in spite of progression of the mucous membrane and osseous lesions; and (6) the failure to develop diabetes insipidus to date. (Arch Dermatol 113:1094-1100, 1977)

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