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A 60-year-old woman with pseudoxanthoma elasticum (PXE) underwent thorough coronary artery disease assessments. Intravascular imaging tests suggested fragmented and calcified elastic fibers in the internal elastic lamina, suggesting a possible pathophysiology of coronary artery disease in PXE. Our case report would allow clinicians to acknowledge the clinical picture of PXE. (Level of Difficulty: Intermediate.).
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