Acute posterior multifocal placoid pigment epitheliopathy

Acute Posterior Multifocal Placoid Pigment Epitheliopathy

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is a rare inflammatory eye disease that affects the retina and the choroid. It is characterized by the sudden onset of multiple, yellow-white placoid lesions at the level of the retinal pigment epithelium (RPE) and the choriocapillaris.

Clinical Presentation

Patients with APMPPE typically present with sudden, painless loss of vision in one or both eyes. The visual impairment can range from mild to severe. Other symptoms may include scotoma, photopsia, and metamorphopsia.

Pathophysiology

The exact cause of APMPPE is unknown, but it is believed to be an inflammatory process involving the choroid and the retinal pigment epithelium. It is thought to be associated with a viral or post-viral immune response. The lesions seen in APMPPE are due to inflammation and blockage of the choriocapillaris, leading to ischemia and damage to the RPE.

Diagnosis

Diagnosis of APMPPE is primarily clinical, supported by imaging studies. Fluorescein angiography typically shows early hypofluorescence of the lesions with late hyperfluorescence. Optical coherence tomography (OCT) may reveal disruption of the outer retinal layers and the RPE.

Treatment

There is no specific treatment for APMPPE, and the condition is usually self-limiting. Most patients experience spontaneous recovery of vision over weeks to months. In some cases, corticosteroids may be used to reduce inflammation, especially if there is significant vision loss or if the condition is bilateral.

Prognosis

The prognosis for APMPPE is generally good, with most patients experiencing significant recovery of vision. However, some patients may have residual visual deficits, such as persistent scotomas or mild visual acuity loss.

Related Conditions

APMPPE is part of a group of white dot syndromes, which includes conditions such as multiple evanescent white dot syndrome (MEWDS), birdshot chorioretinopathy, and serpiginous choroiditis. These conditions share similar clinical features but have distinct differences in their presentation and course.

Related Pages

• Retinal pigment epithelium
• Choroid
• Fluorescein angiography
• Optical coherence tomography

Gallery

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  Acute Posterior Multifocal Placoid Pigment Epitheliopathy

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  Acute posterior multifocal placoid pigment epitheliopathy