Clinical and Experimental Aspects of Xanthomatosis

Abstract

Though in 1873 Fagge mentioned for the first time the complications in the heart and aorta in xanthomatosis and Torok (19) and Arning (2) described cases of sudden death in this condition, only during recent decades has the relation between xanthomatosis of the skin and of the circulatory system been stressed. Of the 11 xanthelasma tuberosum multiplex (x.t.m.) patients we examined since 1930, 6 died suddenly, 3 of them under 20 years, one is living but is suffering from angina pectoris and one was an invalid from claudicatio intermittens for a long time before his cardiac death. Data on the blood chemistry of these patients are given in table I. It is evident that the lipid values are markedly enhanced, the relation between the lipid fraction is in some cases normal, in other abnormal, the blood sugar is always normal. In 1938 Montgomery and Osterberg (8) found in 28 % of their cases of x.t.m. severe cardiovascular damage. About the same time Carl MUller (9) and Thanhauser-Magendantz (18) stressed the fact that xanthoma patients often suffer from angina pectoris or die suddenly. The great frequency of systemic complications in x.t.m. became particularly interesting because of the insight into the pathogenesis of coronary thrombosis and arteriosclerosis that might be gained from this fact. It is well known that it is possible to induce in rabbits xanthoma of the skin and atheromatosis of the aorta by feeding cholesterol to them (18, 15, 17, 5). We undertook similar experiments in order to investigate more closely the blood chemistry under these circumstances. Then we wished to ascertain whether there is a correlation between the various symptoms caused by cholesterol feeding and the different changes observed in the blood. We were interested in the pathological changes to be found in the eyes because an arcus lipoides corneae is often observed in patients with coronary diseases. Various authors deemed this arcus an important symptom and have speculated on the pathological meaning of this disturbance. Finally it was our aim to ascertain if iodine compounds act favorably on this experimental cholesterosis of the rabbit. The reports in the literature on this subject had thus far been rather conflicting, but a favorable influence of iodine on hypertension has been taken for granted. In the cardiologic journals papers on this subject appear regularly. Page (11) claimed in 1952 again that iodine can protect animals against cholesterol deposition. * From the laboratories of the Pharmacological Department (Director Prof. Dr. S. E. de Jongh) and the Dermatological Department (Director Prof. Dr. H. W. Siemens) of the Rijksuniversiteit, Leiden, Holland. t Dermatological department of the Municipal Hospital, The Hague, Holland. Department of Cardiology, University Hospital, Leiden, Holland. Received for publication June 29, 1953.