原发性视网膜色素变性为视网膜色素上皮层变性。该病有常染色体显性遗传,常染色体隐性遗传及性连锁隐性遗传三种类型。其典型特点是:进行性夜盲,视野向心性缩小,晚期可影响中心视力。眼底表现:从赤道部开始出现骨细胞样色素沉着视网膜血管周围色素鞘,视盘呈腊黄色,色素上皮萎缩致使脉络膜血管暴露。荧光素眼底血管造影:可见视网膜血管狭窄,视网膜色素上皮萎缩,晚期病例可见 Primary retinitis pigmentosa degeneration of the retinal pigment epithelium. The disease has autosomal dominant inheritance, autosomal recessive inheritance and sex linked to three types of recessive inheritance. The typical features are: progressive night blindness, decreased visual field to the heart, the late can affect the center of vision. Fundus performance: From the equator began osteocytic pigmented retinal vascular pigment sheath around the disc was wax yellow, pigment epithelium atrophy resulting in choroidal vascular exposure. Fundus fluorescein angiography: visible retinal vascular stenosis, retinal pigment epithelium atrophy, late cases can be seen