eMedicine Specialties > Ophthalmology > Retina

Acute Multifocal Placoid Pigment Epitheliopathy: Differential Diagnoses & Workup

Author: Lakshmana M Kooragayala, MD, Vitreo-retinal Surgeon, Marietta Eye Clinic
Contributor Information and Disclosures

Updated: May 26, 2009

Differential Diagnoses

Multifocal Choroidopathy Syndromes
Neuroretinitis, Diffuse Unilateral Subacute
Sarcoidosis
Toxoplasmosis
Vogt-Koyanagi-Harada Disease
White Dot Syndromes

Other Problems to Be Considered

Serpiginous choroidopathy
Vitiliginous choroidopathy
Acute retinal pigment epitheliitis
Disseminated embolic choroiditis
Focal exudative choroiditis
Hodgkin disease
Other causes of vasculitis (eg, giant cell arteritis, Takayasu disease)

Workup

Laboratory Studies

  • APMPPE is diagnosed from its typical clinical appearance and disease course. No test is pathognomonic or diagnostic for this disease.
  • The following tests may be ordered to help rule out other diseases that may have somewhat similar findings:
    • Antinuclear antibody (ANA)
    • Antineutrophil cytoplasmic antibodies (ANCA)
    • Rheumatoid factor
    • Angiotensin-converting enzyme (ACE)
    • Rapid plasma reagin (RPR)
    • Purified protein derivative (PPD) skin test
    • Anergy panel
    • Chest x-ray
    • Complete blood count (CBC)
    • Sedimentation rate
    • Lyme disease titers
    • Cytomegalovirus (CMV) antibodies
    • Anticardiolipin antibodies

Imaging Studies

  • A CT scan or MRI is indicated in those patients with severe headache or CNS symptoms.
  • A cerebral arteriogram may be indicated when cerebral vasculitis is suspected.

Other Tests

  • Studies that may assist in the diagnosis of APMPPE include the following:
    • Lumbar puncture: Lymphocytic pleocytosis and elevated protein frequently present in spinal fluid if headache is present.
    • Urinalysis: Transient proteinuria, casts, and lymphocytes may indicate a subclinical microvascular nephropathy.
  • Fluorescein angiography
    • Early lesions: Characteristic findings of early hypofluorescence of the lesions are followed by later hyperfluorescence with or without central staining.
    • Older lesions: Such lesions may show window defects in RPE.
  • Indocyanine green (ICG) angiography
    • Early lesions: Numerous round hypofluorescent choroidal defects frequently outnumber those seen on fluorescein angiography. Larger choroidal vessels can be visualized in the hypofluorescent areas, suggesting nonperfusion of choroidal lobules as the source of the typical APMPPE lesions.
    • Older lesions: Partial or complete resolution of the hypofluorescent choroidal areas occurs, and the choroidal findings observed with ICG angiography disappear or resolve earlier than fluorescein angiographic findings. In resolution, the lesions observed with ICG angiography may remain identical to those seen ophthalmoscopically and to the fluorescein angiographic changes.
  • Electroencephalography may show diffuse slowing of wave patterns.
  • Electroretinogram (ERG) findings may be minimally subnormal.
  • Electro-oculogram (EOG) findings may have substantial reduction of light-to-dark ratio studies, which show diffuse functional abnormality of the RPE. Functional recovery may be slow, and, in some instances, it may take up to a year for full recovery.
  • Visual fields may show paracentral scotomata early; some visual defects may be permanent.
  • Dark adaptation may show delayed in the acute phase, which can return to normal with time after recovery from the acute lesions.
  • Optical coherence tomography  
    • In the acute phases, optical coherence tomography (OCT) reveals a mild hyperreflective area above the RPE and, in the later phases, a nodular hyperreflective lesion on the plane of the RPE.
    • These hyperreflective lesions may indicate inflammatory tissue and inflammatory cells or the presence of ischemic edema in the outer retinal layers.
    • Ultra-high resolution OCT of the macula demonstrates early photoreceptor disruption, likely representing early degenerative changes of the photoreceptors. Healed cases continue to demonstrate areas of severe photoreceptor atrophy. Loss of the RPE is also evident during healing and after disease resolution.
  • The Stiles-Crawford effect shows early profound disorientation of the photoreceptors.

Histologic Findings

No histopathology of ocular tissue has been published. Cerebral pathology of a patient with cerebral vasculitis showed granulomatous changes in the vessel wall with giant cells.

More on Acute Multifocal Placoid Pigment Epitheliopathy

Overview: Acute Multifocal Placoid Pigment Epitheliopathy
Differential Diagnoses & Workup: Acute Multifocal Placoid Pigment Epitheliopathy
Treatment & Medication: Acute Multifocal Placoid Pigment Epitheliopathy
Follow-up: Acute Multifocal Placoid Pigment Epitheliopathy
Multimedia: Acute Multifocal Placoid Pigment Epitheliopathy
References
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References

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Further Reading

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Keywords

acute posterior multifocal placoid pigment epitheliopathy, APMPPE, acute multifocal posterior placoid pigment epitheliopathy, AMPPPE, AMPPE, acute placoid pigment epitheliopathy

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Contributor Information and Disclosures

Author

Lakshmana M Kooragayala, MD, Vitreo-retinal Surgeon, Marietta Eye Clinic
Lakshmana M Kooragayala, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, and Medical Association of Georgia
Disclosure: Nothing to disclose.

Medical Editor

Andrew A Dahl, MD, Director of Ophthalmology Teaching, Mid-Hudson Family Practice Institute, The Institute for Family Health; Assistant Professor of Surgery (Ophthalmology), New York College of Medicine
Andrew A Dahl, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

R Christopher Walton, MD, Professor, Director of Uveitis and Ocular Inflammatory Disease Service, Department of Ophthalmology, Assistant Dean for Graduate Medical Education, University of Tennessee College of Medicine; Consulting Staff, Regional Medical Center, Memphis Veterans Affairs Medical Center, St Jude Children's Research Hospital
R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Healthcare Executives, American Uveitis Society, Association for Research in Vision and Ophthalmology, and Retina Society
Disclosure: Nothing to disclose.

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

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