肥厚閉塞性心筋症（H. O. C. M.）を合併した汎発性黒子症候群の1例

A case of the syndrome consisting of lentiginosis associated with hypertrophic obstructive cardiomyopathy (HOCM)

　黒子をはじめとする皮膚症状と，心臓・泌尿生殖器・神経・結合織など全身に亘り多彩な症状を示す汎発性黒子症候群についての報告は本邦では数少なく^1〜12），心障害について詳細に述べられているものは1例^9,10）と思われる。著者らは肥厚閉塞性心筋症に合併した汎発性黒子症候群の1例を経験したので，報告すると共に，文献的考察を加えた。

　A case of the syndrome consisting of lentisinosis associated with cardiac anomalies was reported in a 32 year-old Japanese man. The patient had hypertrophic obstractive cardiomyopathy (HOCM), generalized lentigines, ocular hypertelorism, stravismus convergence and rightside hemiplegia. The diagnosis of the cardiac diesease was established by echocardiography (M-mode and two-dimensional), cardiac catheterization and angiography.

　On reviewing the world literatures concerning the cardiac anomaly of the syndrome, there were 31 reported cases with established diagnosis of the cardiac condition. The most common form of cardiac conditions was HOCM or hypertrophic cardiomyopathy (HCM) which was found in 42%(13/13 cases). The next was pulmonary valvular stenosis found in 32%(10/31 cases). Other cardiac anomalies included 6 cases with pulmonary infundibular stenosis, and one each with dysplastic pulmonary valve and left atrial myxoma.