bilateral acquired abnormality of retinal pigment epithelium and choroid in which irregular multiple progressive swelling is followed by atrophic scars in linear patterns.
Diagnosis Fluorescein angiography, early, active lesion has central hypofluorescence with a hyperfluorescent rim; choriocapillaris and retinal pigment epithelium are absent or attenuated in healed lesions making the larger choroidal vessels easily visible clinically and on angiography
Visual field testing Scotomata, densest at the centre
Management Corticosteroids are used, but don’t stop disease progression, long-term combination therapy with prednisone, azathioprine, cyclosporine
DiffDx Multifocal choroiditis; panuveitis; central areolar choroidal sclerosis; sarcoidosis; syphilitic choroiditis; patients with acute posterior multifocal placoid pigment epitheliopathy (APMPPE), are younger, may have extraocular findings, lesions rarely emanate from optic nerve, acute lesions rarely border inactive scars; age-related macular degeneration; angioid streaks