Epitheliopathy (APMPPE)

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE), also known as acute multifocal posterior placoid pigment epitheliopathy, is a rare eye condition involving the retina and choroid (the layer of blood vessels and connective tissue between the sclera and the retina). It typically affects young adults and is characterized by sudden, but usually temporary, loss of vision.

Symptoms and Signs

The primary symptoms of APMPPE include sudden and often painless vision loss in one or both eyes. Patients may also experience visual field defects, photopsia (flashes of light), and the appearance of scotomas (blind spots). The condition is often preceded by a viral-like illness.

Causes

The exact cause of APMPPE is unknown, but it is thought to be an autoimmune response following a viral infection. This response causes inflammation that affects the choroidal circulation, leading to the characteristic lesions seen in this condition.

Diagnosis

Diagnosis of APMPPE is primarily based on the clinical presentation and fundoscopic findings, which reveal multiple, creamy white lesions at the level of the retinal pigment epithelium (RPE). Ancillary tests such as fluorescein angiography can help in the diagnosis by showing early hypofluorescence and late staining of the lesions. Optical coherence tomography (OCT) and indocyanine green angiography (ICGA) may also be useful in the diagnosis and monitoring of the disease.

Treatment

There is no specific treatment for APMPPE, as the condition is usually self-limiting and tends to resolve on its own within weeks to months. Treatment is mainly supportive and focuses on managing complications, if any. In cases where there is significant vision loss or if the condition does not resolve spontaneously, systemic corticosteroids may be considered to reduce inflammation.

Prognosis

The prognosis for patients with APMPPE is generally good, with most individuals experiencing a significant recovery of vision. However, some patients may have residual visual field defects or other permanent changes in vision.

Epidemiology

APMPPE is a rare condition, and its exact prevalence is unknown. It typically affects young adults in their 20s and 30s, with no clear gender predilection.

Epitheliopathy (APMPPE) Resources
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