Acute zonal occult outer retinopathy in a patient with retinitis pigmentosa.

Abstract

A 30-year-old woman with a phenotypic presentation of retinitis pigmentosa (RP) presented with a 5-day history of painless, acute vision loss in her right eye, with visual acuity dropping from 20/30 to hand motions. Optical coherence tomography of the right macula showed near-complete loss of the ellipsoid layer. Treatment with a prolonged course of oral prednisone resulted in a complete structural regeneration of the ellipsoid layer and improvement of visual acuity to 20/50, with eccentric fixation. Tests for infectious diseases, autoimmune disorders, and rare RP mimic syndromes (eg, Refsum disease) were negative. The patient has remained stable since. We favor a diagnosis of two separate pathologies and suggest a designation of acute zonal occult outer retinopathy (AZOOR) in RP for this previously unreported presentation.