Arcuate pigment epithelial dystrophy

Five cases of an atypical pigmentary retinopathy are described. Findings consisted of a pigment epithelial atrophy occurring in an arcuate fashion, with corresponding visual field and angiographic transmission defects. The pigment epithelial atrophy was subtle, bilateral, and symmetrical. A familial pattern was not present. Electrophysiologic findings were normal or mildly subnormal. The process usually spared the macula. However, two patients demonstrated centrocecal scotomata with an associated significant decrease in visual acuity--a finding not previously described.