(Acute Multifocal Placoid Pigment Epitheliopathy)

BASICS


DESCRIPTION


• Acquired multifocal inflammatory disorder affecting the retinal pigment epithelium (RPE) and choroid


• Frequently bilateral yellowish–white plaques, often in various stages of resolution


EPIDEMIOLOGY


Incidence


Unknown but rare


RISK FACTORS


Genetics


HLA-B7 and HLA-DR2 have been reported with increased frequency


PATHOPHYSIOLOGY


Believed to result from choroidal vascular obstruction


ETIOLOGY


Etiology is unknown but a viral prodrome occurs in one-third of patients


COMMONLY ASSOCIATED CONDITIONS


• Uveitis


• Retinal vasculitis


• Episcleritis


• Erythema nodosum


• Cerebral vasculitis


DIAGNOSIS


HISTORY


• Painless vision loss


• Viral prodrome occurs in one-third of patients


PHYSICAL EXAM


• Funduscopic examination reveals yellowish–white placoid lesions in the macula


– Lesions are typically multiple, bilateral, and in various stages of resolution (resolution is denoted by RPE hyperplasia)


– Lesions reside at the level of the RPE and choroid


DIAGNOSTIC TESTS & INTERPRETATION


Imaging


Fluorescein angiogram shows early hypofluorescence with late hyperfluorescence.


DIFFERENTIAL DIAGNOSIS


• Serpiginous choroiditis


• Relentless/Ampiginous chorioretinitis


• Ocular toxoplasmosis


• Sarcoidosis


• Ocular lymphoma


• Harada disease


TREATMENT


MEDICATION


• Lesions typically resolve without medical treatment


• If lesions affect the fovea and vision is poor, it is reasonable (but unproven) to consider a short course of oral steroids


ADDITIONAL TREATMENT


Issues for Referral


• Suspected AMPPE should be referred to a retinal specialist to aid in diagnosis and care


• Mental status changes should prompt an immediate referral to a neurologist to rule out cerebral vasculitis


ONGOING CARE


FOLLOW-UP RECOMMENDATIONS


• Spontaneous resolution is the rule.


• Recurrence is possible but is rare. When multiple recurrences occur, a different diagnosis should be considered.


Patient Monitoring


Cerebral vasculitis is a rare associated finding. Mental status changes should prompt an immediate referral to a neurologist.


PROGNOSIS


Spontaneous recovery within 3–4 weeks is the norm. In rare instances, RPE changes in the macula can cause chronic vision loss.


ADDITIONAL READING


• Gass JDM. Acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol 1968;80:177–185.


• Heath JD. Acute posterior multifocal placoid pigment epitheliopathy. Int Ophthalmol Clin 1995;35(2):93–105.


• O’Halloran HS, Berger JR, Lee WB, et al. Acute multifocal placoid pigment epitheliopathy and central nervous system involvement: Nine new cases and a review of the literature. Ophthalmology 2001;108(5):861–8.


CODES


ICD9


363.15 Disseminated retinitis and retinochoroiditis, pigment epitheliopathy


CLINICAL PEARLS


• AMPPE is a self-limited inflammatory disease of the RPE and choroid


• Rarely AMPPE may be associated with life-threatening complications such as cerebral vasculitis


• Patients often recover significant visual acuity unless there are significant RPE changes in the fovea


Only gold members can continue reading. Log In or Register to continue

Stay updated, free articles. Join our Telegram channel

Nov 9, 2016 | Posted by in OPHTHALMOLOGY | Comments Off on (Acute Multifocal Placoid Pigment Epitheliopathy)

Full access? Get Clinical Tree

Get Clinical Tree app for offline access