Planar xanthoma

Patient Population. The proband of the B family, T.B., was referred to the Lipid Research Clinic at The Johns Hopkins Hospital at the age of five years because of hypercholesterolemia of 900 mg/100 ml. She had multiple planar xanthomas that had first appeared at three years of age. The patient was free of symptoms of ischemic heart disease. The index lipoprotein pattern was type lib (57), with marked hypercholesterolemia, hyperbeta-lipoproteinemia, a mild hyperprebetalipoproteinemia and hypertriglyceridemia. None of the relatives of T.B. had xanthomas or corneal arcus one (J.S.) developed signs of premature coronary atherosclerosis at the age of 43 years. Increased total plasma and LDL cholesterol levels were transmitted over three generations on both maternal and paternal sides of the family (Fig. I). The parents of the proband, S.B. and K.B., had endogenous hypertriglyceridemia as well. Two normolipidemic members of this family (S.B., Jr. and E.B.), were also studied. 

Patients with type III hyperlipoproteinemia exhibit increased plasma cholesterol and triacylglycerol and the presence of 6-VLDL. Dysbetalipoproteinemics are prone to premature vascular disease, eruptive xanthomas on elbows and knees, and planar xanthomas in the palmar and digital creases. These patients respond well to therapy. Dietary therapy is preferred, but drug therapy (see below) may also be necessary. 

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