Acute posterior multifocal placoid pigment epitheliopathy and cerebral vasculitis

Abstract

Four cases of suspected cerebral vasculitis have been described in association with acute posterior multifocal placoid pigment epitheliopathy, but none was confirmed histopathologically. We describe a patient who developed fatal cerebral vasculitis after placoid pigment epitheliopathy. Histopathologic data revealed a focal granulomatous inflammation of medium-sized arteries in the leptomeninges. Multinucleated giant cells were a conspicuous part of the inflammatory reaction. The cause of both placoid pigment epitheliopathy and cerebral vasculitis remains obscure.