SPECIAL REVIEWS

Abstract

It is suggested that the term "xanthoma" be limited to lesions containing a high percentage of lipid as cholesterol and, since these are small yellow tumors, this seems appropriate. The patient with these lesions deserves careful clinical study, and an estimation of serum lipids. If possible, total serum lipid determination with partition is desirable, especially in the obscure cases. At least an accurate estimation of serum cholesterol is needed before a logical prognosis and course of treatment can be provided. The Schoen-heimer-Sperry cholesterol method has the widest acceptance. Patients with xanthomas can be divided into those with normal serum lipids and those with elevated lipids. A. Xanthoma disseminatum lesions are the most frequent and the most benign, and are associated with normal serum cholesterol values. B. Xanthomas without elevation of serum lipids are also found in patients with the "eosinophilic granuloma syndromes," and occasionally with other chronic diseases. C. In xanthoma tuberosum the serum cholesterol is markedly elevated, and the frequent cardiac involvement gives the full-blown patient a poor prognosis. D. Secondary or eruptive xanthomas can accompany most of the conditions with chronic hyperlipemia irrespective of the cause, but they are most common in diabetes mellitus and idiopathic familial hyperlipemia. Another case of idiopathic familial hyperlipemia in an infant is reported, with a total serum lipid level of 6.2%, as well as two cases of the nephrotic syndrome in childhood with secondary xanthomas, a case of xanthomas complicating chronic obstructive liver disease, an example of eruptive xanthomata in von Gierke's disease, and a description of a diabetic girl with a serum total lipid level of 19.4%. The basic cause for the accumulation of cholesterol in any of these conditions is unknown. Surgical removal of the lesions is rarely advisable, and dietary treatment is indicated only in certain of the hypercholesterolemic syndromes.