PRIMARY XANTHOMA TUBEROSUM IN CHILDREN

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Abstract
A brief classification of xanthomatoses is presented. In an attempt to remove some of the confusion contributed by the several different meanings for the term "hyperlipemia," the term "hyperneutralipemia" is introduced to indicate an elevation of the concentrations of neutral fat in the serum. The term "hyperlipemia" is retained to mean an elevation of values for any of the blood lipids. The term "primary xanthoma tuberosum" designates the classic idiopathic form of xanthoma tuberosum associated with hypercholesteremia and without hyperneutralipemia. The primary purpose of this paper is to review the outcome of 11 cases of primary xanthoma tuberosum in children seen at the Mayo Clinic. Five of the eleven are known to be dead from cardiovascular disease, and one other is presumed to be dead. In five of the six patients angina pectoris developed before death. Two patients who are still living have angina pectoris, one having suffered myocardial infarction. Necropsy of four patients showed atheromatous involvement of the coronary blood vessels to be a prominent finding. Three of the patients died before they reached the age of 15 years. Two of the patients were brother and sister. Of the remaining nine patients, four gave a family history of cardiac disease occurring in a close relative early in life and five patients had relatives with cutaneous xanthomas. In the one family studied, all five members had hypercholestenemia.

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