本文报告一例以前从未有文献记载的视网膜脉络膜变性伴发进行性虹膜坏死。患者男性,34岁,1986年7月开始感双眼疼痛及中心视力下降。一个月后就诊,全身体检无异常,双眼视力均为20/60(2年前为20/20),眼部检查发现黄斑区有异常的视网膜色素上皮(RPE)非特异性斑驳。此后视力进行性下降。发病后5个月视力降至20/300。1987年3月眼底血管荧光造影显示RPE斑驳较前更严重,黄斑及RPE萎缩区有小的荧光窗样 This article reports a case of retinal choroidal degeneration with progressive iris necrosis that has never been previously documented. The patient, male, 34 years old, began to feel binocular pain and decreased central vision in July 1986. Visits one month later, no abnormal physical examination, binocular visual acuity was 20/60 (20/20 2 years ago), ocular examination found abnormal macular retinal pigment epithelium (RPE) non-specific mottle. After vision decreased. Five months after onset of vision decreased to 20 / 300. 1987 March fundus fluorescein angiography RPE mottled more serious than before, macular and RPE atrophy with a small fluorescent window-like