Xanthoma disseminatum is a normolipemic disseminated xanthomatosis that tends to localize on the flexural and intertriginous surfaces, is often associated with diabetes insipidus, and appears to run a chronic benign course. Xanthomatous deposits may also be found on the mucous membranes of the mouth, pharynx and upper respiratory tract, and on the conjunctiva and cornea. Dermal infiltration by a mixture of cell types including histiocytes, xanthoma cells, numerous Touton giant cells, and inflammatory cells is typical. Lesions may persist indefinitely or involute spontaneously after many years. Respiratory obstruction due to xanthomatous involvement is a rare, serious complication and may require tracheotomy.
The disease is believed to be a granulomatous histiocytic proliferation in which lipid accumulation is secondary and is unassociated with systemic disturbances of lipid metabolism or transport. It appears to be one expression of a broad spectrum of histiocytic proliferative disorders including histiocytosis X, juvenile xanthogranuloma, histiocytoma, and reticulohistiocytoma. Usually these entities are distinct and readily categorized with their existing terminology. Observation of rare cases presenting merging clinicopathologic patterns enables one to appreciate the basic similarities underlying all these disorders.
We present a review of world literature on xanthoma disseminatum and summarize the clinicopathologic features and course in 7 Mayo Clinic cases.
ALTMAN J, WINKELMANN RK. Xanthoma Disseminatum. Arch Dermatol. 1962;86(5):582–596. doi:10.1001/archderm.1962.01590110018003
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: