Acute Zonal Occult Outer Retinopathy (AZOOR) and Related Diseases.

Acute zonal occult outer retinopathy (AZOOR) is a presumed inflammatory disorder with outer retinal dysfunction. Typically, the onset is acute and it is unilateral, with symptoms of photopsias and nasal field loss; scotoma is usually contiguous with the optic nerve. Later, the other eye is involved in nearly three fourths of patients. The central vision remains good in most cases. Patients are usually young women with myopia. Fundus: May be normal in the beginning, but may show a grayish-white line at the border of normal and involved retina, usually in peripapillary area. This line disappears within weeks and is replaced with an orange zone. With time, retinal vessels attenuate and a large zone of retinal pigment epithelium (RPE) depigmentation appears, sort of a sector retinitis pigmentosa (RP) or unilateral or asymmetric RP. Rarely, mild vitritis may occur, and relative afferent pupillary defect (RAPD) is present in about 75% of cases.