eMedicine Specialties > Ophthalmology > Retina

Acute Multifocal Placoid Pigment Epitheliopathy: Treatment & Medication

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

Updated: May 26, 2009

Treatment

Medical Care

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.

Surgical Care

In cases complicated by subretinal neovascularization, laser photocoagulation may be useful.

Consultations

Consultations may be indicated if the diagnosis is not clear or if a systemic manifestation indicates such a need (an infrequent occurrence).

  • Neurologist/neurosurgeon - CNS symptoms
  • Urologist - Urinalysis findings
  • Dermatologist - Skin findings
  • Rheumatologist - Serum immunologic abnormalities
  • Infectious disease specialist

Diet

No dietary restrictions are indicated.

Activity

No limitations of visual or physical activities are indicated unless systemic manifestations impose limitation of physical activities.

Medication

In most cases, the lesions resolve spontaneously, and no therapy is required. Some authors have used corticosteroids to treat the ocular disease and/or any severe systemic manifestations. However, there is no evidence that treatment with corticosteroids affects the visual outcome in patients with APMPPE. Various routes of administration (eg, topical, oral, pulse intravenous, sub-Tenon injection) and dosages of corticosteroids have been used. Cycloplegics may be useful for severe iritis, an infrequent finding.

Corticosteroids

Suppress ocular and systemic inflammation.


Prednisone (Deltasone, Orasone, Meticorten)

May be indicated when signs of systemic vasculitis are present, given either orally or by pulse IV therapy. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Adult

Initially, 40-60 mg/d PO in single or divided dose, taper as indicated; up to 1 g methylprednisolone infused in solution over half-hour period; topically, 1 gtt up to qh while awake

Pediatric

Up to 2 mg/kg/d PO, depending upon indication and severity

Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Documented hypersensitivity; microbial infection (particularly tuberculosis); peptic ulcer disease; hepatic dysfunction; use with caution in patients with diabetes, hypertension, congestive heart failure, and renal failure

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

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

References

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

  2. Bugnone AN, Hartker F, Shapiro M, Pineless HS, Velez G. Acute and chronic brain infarcts on MR imaging in a 20-year-old woman with acute posterior multifocal placoid pigment epitheliopathy. AJNR Am J Neuroradiol. Jan 2006;27(1):67-9. [Medline][Full Text].

  3. Di Crecchio L, Parodi MB, Saviano S, Ravalico G. Acute posterior multifocal placoid pigment epitheliopathy and ulcerative colitis: a possible association. Acta Ophthalmol Scand. Jun 2001;79(3):319-21. [Medline].

  4. Howe LJ, Woon H, Graham EM, Fitzke F, Bhandari A, Marshall J. Choroidal hypoperfusion in acute posterior multifocal placoid pigment epitheliopathy. An indocyanine green angiography study. Ophthalmology. May 1995;102(5):790-8. [Medline].

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

  6. Lim LL, Watzke RC, Lauer AK, Smith JR. Ocular coherence tomography in acute posterior multifocal placoid pigment epitheliopathy. Clin Experiment Ophthalmol. Nov 2006;34(8):810-2. [Medline].

  7. Lofoco G, Ciucci F, Bardocci A, Quercioli P, Steigerwalt RD Jr, De Gaetano C. Optical coherence tomography findings in a case of acute multifocal posterior placoid pigment epitheliopathy (AMPPPE). Eur J Ophthalmol. Jan-Feb 2005;15(1):143-7. [Medline].

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  9. Mensah E, Vafidis GC. Acute posterior multifocal placoid pigment epitheliopathy in a 7-year-old girl. J Pediatr Ophthalmol Strabismus. Jul-Aug 2002;39(4):239-41. [Medline].

  10. O'Halloran HS, Berger JR, Lee WB, Robertson DM, Giovannini JA, Krohel GB, et al. Acute multifocal placoid pigment epitheliopathy and central nervous system involvement: nine new cases and a review of the literature. Ophthalmology. May 2001;108(5):861-8. [Medline].

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  14. Smith CH, Savino PJ, Beck RW, Schatz NJ, Sergott RC. Acute posterior multifocal placoid pigment epitheliopathy and cerebral vasculitis. Arch Neurol. Jan 1983;40(1):48-50. [Medline].

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  17. Wolf MD, Folk JC, Panknen CA, Goeken NE. HLA-B7 and HLA-DR2 antigens and acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol. May 1990;108(5):698-700. [Medline].

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Further Reading

Keywords

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

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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