Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)

Abstract

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) like other inflammatory entities of the choriocapillaris was included in the purely descriptive pot-pourri group of white dot syndromes, a classification that nowadays has no reason to be maintained. As for many fundus diseases, it was J. Donald M. Gass who first described the disease in 1968. He called the disease acute posterior multifocal placoid pigment epitheliopathy (APMPPE) because he thought that the disease was due to an acute cellular response on the part of the retinal pigment epithelium (RPE) to a local noxious agent. In 1971, van Buskirk et al. suggested choriocapillaris perfusion as the underlying disorder. In 1972 and again in 1977 and 1983, Deutman and colleagues, based on the choriocapillaris nonperfusion seen on early FA frames, indicated that it was the choriocapillaris rather than the RPE that was primarily involved, and they suggested to rename the disease as “acute multifocal ischaemic choriocapillaropathy” (AMIC). With the aid of indocyanine green angiography (ICGA), Dhaliwal et al. identified choriocapillaris nonperfusion as the primary event in APMPPE.

APMPPE predominantly occurs in young individuals during the second to fourth decade of life.