心内膜に著明な線維増生を認め，拘束型心筋症を呈した弾力線維性仮性黄色腫（Grönblad-Strandberg症候群）の1例

A Case of Pseudoxanthoma Elasticum (Grönblad-Strandberg Syndrome) with Marked Subendocardial Fibrosis and Secondary Restrictive Cardiomyopathy

　弾力線維性仮性黄色腫（以下本症）は全身の弾力線維の変性を来し，特有の皮膚変化と眼底所見，心血管系合併症を起こす稀な遺伝性疾患である．われわれは動悸を主訴に受診した弾力線維性仮性黄色腫の1例を経験した．症例は62歳の女性で，高血圧，狭心症，消化管出血，糖尿病，甲状腺自己免疫異常を合併していた．心内膜下心筋生検で弾性線維の変性と著明な線維増生を認めた．心収縮能は良好であったが，左室拡張障害によって続発性拘束型心筋症を呈していた．本邦では本症に伴う拘束型心筋症の報告は稀であるが，拘束型心筋症の病態を呈する場合は本症も念頭に置く必要がある．

Pseudoxanthoma elasticum (PXE) is a rare heredi-tary disorder characterized by systemic degeneration of elastic fibers which induces skin lesions, fundus lesions and cardiovascular complications. A 62-year-old woman who complained of palpitations was admitted to our hospital. She was diagnosed as having PXE complicated by hypertension, angina pectoris, gastro-intestinal bleeding, diabetes mellitus and autoimmune disturbance of the thyroid gland. A subendocardial biopsy showed degeneration of elastic fibers and marked fibrosis. Although left ventricular systolic function of the patient's heart was relatively good, reduction in her left ventricular compliance caused secondary restrictive cardiomyopathy (RCM). Although PXE accompanying RCM has not been report-ed in Japan, this possibility should be considered when a patient develops RCM.