Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)

Updated: May 03, 2017
  • Author: Lakshmana M Kooragayala, MD; Chief Editor: C Stephen Foster, MD, FACS, FACR, FAAO, FARVO  more...
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Overview

Background

First described by Gass in 1968, acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an acquired inflammatory disorder affecting the retina, retinal pigment epithelium, and choroid of otherwise young healthy adults. [1] The disease is self-limited and is characterized by multiple yellow-white placoid subretinal lesions of the posterior pole, as shown below. The lesions are frequently bilateral and in various stages of evolution, typically resolving in weeks to months and leaving circumscribed areas of retinal pigment epithelial disturbance.

Posterior pole of right eye. Early acute posterior Posterior pole of right eye. Early acute posterior multifocal placoid pigment epitheliopathy lesion shows yellowish-white placoid lesion involving the macula and an area just inferior temporal to the macula.
Posterior pole of left eye of same patient showing Posterior pole of left eye of same patient showing acute posterior pole placoid lesion.
Inferior nasal of right eye of the same patient ap Inferior nasal of right eye of the same patient approximately 2 months later, showing scattered areas of retinal pigment epithelium atrophy and hyperplasia.

Visual acuity may be affected significantly if the macula is involved, but recovery is the rule with or without systemic therapy, with 80% of those eyes affected achieving a visual acuity of 20/40 or better. The cause is unknown but believed to be a hypersensitivity-induced vasculitis involving not only terminal choroidal lobules but also diffuse systemic large and small vessels.

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Pathophysiology

The pathophysiology of APMPPE is somewhat speculative.

Two major theories concerning the pathogenesis of APMPPE have been proposed based on clinical and diagnostic findings. No histopathological evidence is available on acute or subacute stages of the disease.

The first suggests that inflammation of the retinal pigment epithelium (RPE) and outer retina manifests as placoid lesions during the acute phase. [1]

The second theory, which is more convincing, suggests that choroidal vasculitis leads to partial occlusion of the choriocapillaris in these eyes. In turn, disruption of choroidal blood flow causes secondary ischemia of the overlying RPE and outer retina, manifesting as placoid lesions. [2]

Other findings suggestive of vasculitis include episcleritis, erythema nodosum, microvascular nephropathy, thyroiditis, optic neuritis, labyrinthitis, hearing loss, and cerebral angiitis. Meningoencephalitis occasionally has been observed.

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Epidemiology

Frequency

United States

The incidence and prevalence of APMPPE is unknown. However, since the landmark description of Gass, APMPPE has been reported frequently and widely from ophthalmic centers, primarily in the United States and Western Europe. Most US patients reported in the literature reside in the northern and midwestern states.

International

Reports in the international literature have included patients from northwestern European countries, such as England, Scotland, France, Belgium, the Netherlands, and Denmark. Several reports have originated from Japan. Reports from other geographic areas have been sparse.

Mortality/Morbidity

APMPPE usually affects healthy adults, and, other than ocular involvement, systemic manifestations are relatively uncommon. When they do occur, many systemic manifestations are usually mild and transient in nature; however, the presence of cerebral vasculitis has been associated with permanent neurologic sequelae, such as hemiparesis and even death from intracerebral edema and brain herniation in rare instances.

Race

Almost 80% of the recorded cases include whites, with the remainder being Japanese, African American, Nepalese, and from the Indian subcontinent. Whether this racial distribution represents a predilection of APMPPE for whites or a reporting bias is unclear.

Sex

Earlier reports of APMPPE suggested a slight preponderance of women with this disease, but more recent publications suggest no sexual predilection, with equal frequency between both men and women.

Age

The mean age of onset is approximately 27 years. The documented age range of onset is 7-66 years. The most frequent age range of occurrence of APMPPE is in those patients aged 16-40 years, representing approximately 85% of cases. About 50% of patients present in the third decade of life.

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