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We studied four patients with peripapillary pigmentary retinal degeneration, an asymptomatic disorder that was probably benign and nonprogressive. The ophthalmoscopic appearance showed a segmental, grayish metallic sheen in association with bone spicule pigmentation, which radiated from the disk along the temporal vessel arcades and joined temporal to the macula. The optic disk, retinal vessels, periphery, and macula were normal in all patients. Visual function tests and fluorescein angiography indicated a segmental disease of the retinal pigment epithelium-photoreceptor complex.
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