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Article
January 1942

HEREDITARY XANTHOMATOSIS: FAMILIAL INCIDENCE OF XANTHOMA TUBEROSUM ASSOCIATED WITH HYPERCHOLESTEREMIA AND CARDIOVASCULAR INVOLVEMENT, WITH REPORT OF SEVERAL CASES OF SUDDEN DEATH

Author Affiliations

NEW YORK; WILKES-BARRE, PA.

From the Skin and Cancer Unit of the New York Post-Graduate Medical School and Hospital and the Wilkes-Barre General Hospital.

Arch Derm Syphilol. 1942;45(1):1-18. doi:10.1001/archderm.1942.01500070005001
Abstract

In accordance with the classification accepted by Thannhauser and Magendantz,1 Montgomery2 and other investigators, primary essential xanthomatosis may be divided into two main types, xanthoma tuberosum and xanthoma disseminatum. The distinction between these forms rests on a variety of clinical and biochemical differences, which concern the cholesterol content of the blood as well as the type and localization of the cutaneous and visceral lesions.

In xanthoma disseminatum there are numerous fine papules and plaques on the flexor surfaces of the extremities, especially in the axillary folds. The mucous membranes of the mouth, pharynx and larynx are often affected, and as a result of involvement of the pituitary gland there is frequent occurrence of diabetes insipidus. Thannhauser and Magendantz also associated with this disease involvement of the bones, lungs and brain and Schüller-Christian disease. The cholesterol content of the blood is usually normal in patients with this disease, which has been designated, therefore,

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