Successful revascularization of advanced coronary artery disease associated with pseudoxanthoma elasticum

Pseudoxanthoma elasticum (PXE) is caused by loss-of-function mutations of the ATP-binding cassette subfamily C member 6 gene. A 58-year-old man was diagnosed as having PXE based on typical findings in orbital and skin biopsies. Coronary computed tomography (CT) showed severe coronary stenosis in the proximal right coronary artery (RCA), and chronic total occlusion (CTO) of the mid left anterior descending coronary artery (LAD) with bridging collaterals. Coronary angiography revealed 99% stenosis in the RCA (#1) and CTO in the mid LAD (#7) with well-developed collaterals from the LAD to the RCA. We performed percutaneous coronary intervention (PCI) and achieved complete revascularization. Intravascular ultrasound (IVUS) showed a superficial high echoic component around the vessels throughout the length of coronary arteries including non-stenotic regions. In the IVUS findings, the main cause of stenosis of the RCA lesion was large amounts of plaque, and the cause of the CTO in the LAD was coronary negative remodeling. In this case, coronary CT was clinically useful in the identification of ischemic heart disease. Since IVUS demonstrates variable findings in each coronary artery lesion and the morphologic characteristics might alter the strategy of PCI, IVUS should be performed at the time of PCI in PXE patients. <Learning objective: Pseudoxanthoma elasticum (PXE) has higher incidence of coronary artery disease (CAD). Coronary computed tomography is clinically useful in the identification of CAD in PXE patients. Intravascular ultrasound should be performed at the time of percutaneous coronary intervention in PXE patients, since the stenotic lesions of PXE have some morphological variations with a superficial high echoic component.>.