Acute Posterior Multifocal Placoid Pigment Epitheliopathy

Definition

Definition of Acute Posterior Multifocal Placoid Pigment Epitheliopathy
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is a chorioretinal disease in which light-coloured lesions begin to form in the posterior pole and inside the retina of the eye. Over time, the lesions cause vision loss that can go from mild to severe, but with no pain involved. Classified as an inflammatory disorder that is acute, but self-limiting, the lesions leave behind scarring, but visual acuity eventually improves.

Symptoms

Symptoms of Acute Posterior Multifocal Placoid Pigment Epitheliopathy
General symptoms include:

1. Blurred vision
2. Scotomas
3. Photopsia
4. Metamorphopsia

Causes

Causes of Acute Posterior Multifocal Placoid Pigment Epitheliopathy
The origin of the infection remains unknown, with various theories of it occurring as an immune response to a larger infection, or the possibility of it being viral because of the preceding flu-like illness that generally accompanies it.

Diagnosis

Diagnosis of Acute Posterior Multifocal Placoid Pigment Epitheliopathy
The diagnosis is made via clinical presentation and fundoscopic examination. Fluorescein angiography is helpful in confirming the diagnosis.

Treatment

Treatment of Acute Posterior Multifocal Placoid Pigment Epitheliopathy
The treatment of APMPPE is somewhat controversial; however, the consensus is that no treatment seems to alter the course of the ocular lesions. The fundus lesions appear to run a relatively short self-limited course, which probably results from a one-time insult to the capillaries comprising the choroidal lobules.

Prognosis

Prognosis of Acute Posterior Multifocal Placoid Pigment Epitheliopathy
APMPEE is self-limiting and it is believed that most patients achieve a visual acuity of 20/40 or better. However; a recent review by Fiore Telal. looked at 183 articles related to APMPPE and concluded that visual outcomes are not as favorable as previously thought with 25% of patients having a visual acuity at 20/50 or worse. There is no current consensus on treatment although steroids have been used and reported to be beneficial in cases of foveal involvement and associated CNS vasculitis. Future studies are needed to evaluate dosage, duration, and effects of steroid treatment. All patients with a new diagnosis a APMPPE should receive a full neurologic and systemic work-up to evaluate for CNS vasculitis and other associated systemic conditions.