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JULES ALTMAN, M.D.; R. K. WINKELMANN, M.D., Ph.D.

Arch Dermatol. 1962;86(5):582-596.

Abstract
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.

Author Affiliations
ROCHESTER, MINN.

Fellow in Dermatology (Dr. Altman), Mayo Foundation; Section of Dermatology (Dr. Winkelmann), Mayo Clinic and Mayo Foundation.

Footnotes
Abridgment of thesis submitted by Dr. Altman to the Faculty of the Graduate School of the University of Minnesota in partial fulfillment of the requirements for the degree of Master of Science in Dermatology.

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