RESUMO
Paravenous pigmented chorioretinal atrophy (PPRCA) is a generally multifocal, bilateral and symmetric rare entity associated with autoimmune diseases and other ocular complications. We present the clinical case of a patient with rheumatoid arthritis who attended for pain of several days. He presented decreased visual acuity of the left eye (LE), nodular scleritis and chorioretinal atrophy with pigment accumulation in bone spicules in the inferior temporal vascular arcade and lamellar macular hole (AML). The right eye shows no alterations. LE autofluorescence (AF) shows a hypoautofluorescence lesion with defined edges. Fluorescein angiography (FAG) shows hyperfluorescence consistent with retinal pigmentary epithelial degeneration and blockage in pigment areas. The visual field (VC) reveals a defect in the superior hemifield. This case describes an atypical unifocal and unilateral PPRCA. This variant must be known to make a correct differential diagnosis, as well as to provide adequate prognostic information.