冠動脈に有意狭窄病変を認めない急性心筋梗塞を発症した弾力線維性仮性黄色腫の1例

A case of pseudoxanthoma elasticum associated with myocardial infarction which revealed absence of significant narrowing of caronary artery

　弾力線維性仮性黄色腫（pseudoxanthoma elasticum：PXE）は，弾力線維の変性・石灰化を来たす遺伝性疾患で，特有の皮膚症状と眼底のangioid streakを伴い，心血管障害の合併の重要性が報告されている。今回，われわれは急性心筋梗塞を発症し，冠動脈造影上，有意な狭窄病変を認めなかった，PXEの1症例を経験したので，本症の心血管障害について若干の文献的考察を加えて報告する。

Pseudoxanthoma elasticum (PXE) is a rare disease that indicates general elastic fiber degeneration, calci-fication and cardiovascular changes. In cardiovascular invol ement, angina pectoris was reported 19% in PXE. Although angina pectoris is frequent, myocar-dial infarction is reported as being uncommon. We reported a case of a-58-year-old woman with typical skin manifestation of PXE confirmed by skin biopsy who presented classical electrocardiographic sign of anteroseptal myocardial infarction.

But coronary angiography showed no significant narrowing of coronary artery and endmyocardial biopsy showed no abnormalities.

Myocardial infarction with PXE in our case seemed to be not a change induced to PXE, but spasm on the basis of bronchial asthma.