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

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