Xanthomatosis and other clinical findings in patients with elevated levels of very low density lipoproteins

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Abstract
Patients [46] with xanthomatosis and elevated very low density lipoproteins (VLDL) levels (in different types of hyperlipoproteinemia) were classified on the basis of the WHO criteria and the cholesterol/triglyceride ratio in VLDL. A large majority (31/46) of the patients could be classified as hyperlipoproteinemia type III, only 8/46 as type IIB and 7/46 as type IV/V. This distinction seems to be relevant as the xanthomatous lesions differed distinctly between these three types of hyperlipoproteinemia. Xanthochromia striata palmaris was present in 29/31 cases of hyperlipoproteinemia type III and was not found in type IV/V patients, who had distinctive papuloeruptive xanthomas. During a follow-up in 35/46 patients all xanthomas disappeared within 2 yr except the xanthelasma palpebrarum and tendinous xanthomas. All type IV/V patients (7/7) but only 1 type III patient (1/31) had abnormal glucose tolerance. Only 2/18 type III patients less than 45 yr showed claudication and none of the young type III patients had angina pectoris. All 4 type IIB patients less than 45 yr had clinical signs of atherosclerosis. Angina pectoris and/or claudication were present in 5/13 type III patients over 45 yr old. The mean serum cholesterol level was equally elevated in both groups but the cholesterol was mainly present in VLDL in type III and in low density lipoproteins (LDL) in type IIB. In 9/31 type III patients the LDL level was also elevated but was easily normalized by a diet low in carbohydrate; the elevated LDL level in type IIB was therapy-resistant. The recognition of xanthomatous lesions, specifically xanthochromia striata palmaris, as an early sign of type III hyperlipoproteinemia, can lead to the early diagnosis and successful treatment of these patients, and thus possibly prevent the development of premature atherosclerosis.