Acute Posterior Multifocal Placoid Pigment Epitheliopathy

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Original article contributed by: Matej Polomsky, MD
All contributors: Elan002 and WikiWorks
Review: Not reviewed
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Article summary goes here.

Disease Entity[edit | edit source]

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an inflammatory chorioretinopathy which was first described by Gass in 1968. It is classified as a White Dot Syndrome. APMPPE is usually bilateral, affects women and men equally, has a tendency to occur in the 3rd to 4th decades, and can have associated systemic conditions. The most common complaint is blurred vision with central or paracentral scotomas. Cases are often self-limited and visual symptoms resolve by 4 to 8 weeks. There is no consensus on treatment, however steroids have been attempted to hasten visual recovery especially in cases with macular involvement.

Etiology[edit | edit source]

The exact etiology is unknown, but several associations have been reported including HLA-B7 and HLA-DR2 genetic haplotypes. Approximately 33% of patients have a preceding viral illness prior to symptom onset. APMPPE has also been described in cases of thyroiditis, erythema nodosum, Wegener's granulomatosis, polyarteritis nodosa, nephritis, sarcoidosis, and CNS vasculitis. Other infectious associations include Lyme disease, mumps, and tuberculosis.

General Pathology[edit | edit source]

The exact pathogenesis is controversial but is believed to be inflammation at the level or the choriocapillaris leading to hypoperfusion and ischemia of the RPE and photoreceptors. In later stages, the inflamed choroid and retina is replaced with RPE atrophy and hyperpigmentation.

Primary prevention[edit | edit source]

There are no recognized preventative measures.

Diagnosis[edit | edit source]

The diagnosis is made via clinical presentation and fundoscopic examination. Fluorescein angiography is helpful in confirming the diagnosis.

25 year-old with multiple yellow-white placoid lesions OD.

History[edit | edit source]

Patients typically notice a rapid onset of blurred vision associated with central and paracentral scotomas. Photopsias have been reported prior to vision loss. The symptoms are usually bilateral, asymmetric and can occur several days apart. Visual acuity can range from 20/40 to count-fingers depending on the extent of foveal involvement.

Physical examination[edit | edit source]

Anterior segment exam is usually normal and there may be mild vitritis present in 50% of the cases. Fundoscopic examination typically shows multiple bilateral yellow-white placoid lesions at the level of RPE and choroid 1-2 disc diameters throughout the posterior pole to the equator. The lesions gradually fade over the course of 1-2 weeks. New lesions may appear in the periphery up to 3 weeks following onset (radially or linearly). Older lesions are replaced with RPE atrophy or hyperpigmentation.

Diagnostic procedures[edit | edit source]

No specific laboratory tests exist to confirm the diagnosis. Fluorescein angiography and Indocyanine Green angiography can be performed which show the following characteristic findings:

Fluorescein angiogram (FA) - early hypofluorescence (blockage) corresponding to the placoid lesions followed by later hyperfluorecent staining

Indocyanine Green (ICG) angiogram - early and late hypofluorescence corresponding to the placoid lesions

Fluorescein angiography: early hypoflurorescence.
Fluorescein angiography: later hyperflurorescence.

Differential diagnosis[edit | edit source]

Other White-Dot syndromes can resemble APMPPE. These include, but are not limited to, multiple evanescent white dot syndrome, serpiginous choroiditis, multifocal choroiditis and panuveitis, punctate inner choroidopathy, and birdshot chorioretinopathy. Other infectious uveitis and lymphoma may present with placoid lesions as well and should be ruled out with appropriate tests if clinical suspicion high.

Prognosis and General Management[edit | edit source]

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 T el al. 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.

References[edit | edit source]

1. Burés-Jelstrup A, Adán A, Casaroli-Marano R. Acute posterior multifocal placoid pigment epitheliopathy. A study of 16 cases. Arch Soc Esp Oftalmol. 2007 May;82(5):291-7

2. Hsu CT, Harlan JB, Goldberg MF, Dunn JP. Acute posterior multifocal placoid pigment epitheliopathy associated with a systemic necrotizing vasculitis. Retina. 2003 Feb;23(1):64-8.

3. Quillen DA et al. The white dot syndromes. Am J Ophthalmol. 2004 Mar;137(3):538-50

4. Comu S, Verstraeten T, Rinkoff JS, Busis NA. Neurological manifestation of acute posterior multifocal placoid pigment epitheliopathy. Stroke. 1996 May;27(5):996-1001

5. O’Halloran HS et al. Acute multifocal placoid pigment epitheliopathy and central nervous system involvement: nine new cases and a review of the literature. Ophthalmology. 2001 May;108(5):861-8

6. Fiore T el al. Acute posterior multifocal placoid pigment epitheliopathy: outcome and visual prognosis. Retina. 2009 Jul-Aug;29(7):994-1001

7. Retina and Vitreous, Section 12. Basic and Clinical Science Course, AAO, 2009-10.

8. Gass JD. Acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol. 1968 Aug;80(2):177-85

9. Jones NP. Acute posterior multifocal placoid pigment epithliopathy. Br J Ophthalmol. 1995 Apr;79(4):384-9