NEW REPORT OF SEVERE CORONARY-ARTERY DISEASE IN AN 18-YEAR-OLD GIRL WITH PSEUDOXANTHOMA ELASTICUM - CASE-REPORT AND REVIEW OF THE LITERATURE

An eighteen-year-old woman with pseudoxanthoma elasticum (PXE) suffere d from mild angina pectoris over a ten-year period. Severe triple-vess el disease with mild left ventricular dysfunction was demonstrated on angiography. No revascularization was feasible. Despite a reported hig h frequency of angina pectoris among patients with PXE, only 10 convin cing reports have appeared in the literature. Careful coronary artery evaluation is required in young patients with PXE, even though asympto matic, because coronary artery disease (CAD) seems to be frequent and because no precise feature can be distinguished between types with or without severe vascular disease. Through very rare reports, surgical r evascularization appears feasible and beneficial in a less severe form of CAD in patients with PXE. The risk of premature and severe diffuse CAD in PXE does not seem to be explained only by the combination of i ncreased Lp [a] (or any other risk factor) and PXE.