Acute Multifocal Placoid Pigment Epitheliopathy 

  • Author: Lakshmana M Kooragayala, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Aug 4, 2011
 

Background

First described by Gass in 1968, acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an acquired inflammatory disorder affecting the retina, retinal pigment epithelium, and choroid of otherwise young healthy adults.[1] The disease is self-limited and is characterized by multiple yellow-white placoid subretinal lesions of the posterior pole, as shown below. The lesions are frequently bilateral and in various stages of evolution, typically resolving in weeks to months and leaving circumscribed areas of retinal pigment epithelial disturbance.

Posterior pole of right eye. Early acute posteriorPosterior pole of right eye. Early acute posterior multifocal placoid pigment epitheliopathy lesion shows yellowish-white placoid lesion involving the macula and an area just inferior temporal to the macula. Posterior pole of left eye of same patient showingPosterior pole of left eye of same patient showing acute posterior pole placoid lesion. Inferior nasal of right eye of the same patient apInferior nasal of right eye of the same patient approximately 2 months later, showing scattered areas of retinal pigment epithelium atrophy and hyperplasia.

Visual acuity may be affected significantly if the macula is involved, but recovery is the rule with or without systemic therapy, with 80% of those eyes affected achieving a visual acuity of 20/40 or better. The cause is unknown but believed to be a hypersensitivity-induced vasculitis involving not only terminal choroidal lobules but also diffuse systemic large and small vessels.

Next

Pathophysiology

The pathophysiology of APMPPE is somewhat speculative, but the basic underlying mechanism is believed to be an obstructive vasculitis, causing nonperfusion of the terminal choroidal lobules in the posterior pole of the eye and inducing secondary ischemic injury of the overlying retinal pigment epithelium and neuroreceptors, which may be partially transient in nature with variable recovery of function.

Other findings suggestive of vasculitis include episcleritis, erythema nodosum, microvascular nephropathy, thyroiditis, optic neuritis, labyrinthitis, hearing loss, and cerebral angiitis. Meningoencephalitis occasionally has been observed.

Previous
Next

Epidemiology

Frequency

United States

The incidence and prevalence of APMPPE is unknown. However, since the landmark description of Gass, APMPPE has been reported frequently and widely from ophthalmic centers, primarily in the United States and Western Europe. Most US patients reported in the literature reside in the northern and midwestern states.

International

Reports in the international literature have included patients from northwestern European countries, such as England, Scotland, France, Belgium, the Netherlands, and Denmark. Several reports have originated from Japan. Reports from other geographic areas have been sparse.

Mortality/Morbidity

APMPPE usually affects healthy adults, and, other than ocular involvement, systemic manifestations are relatively uncommon. When they do occur, many systemic manifestations are usually mild and transient in nature; however, the presence of cerebral vasculitis has been associated with permanent neurologic sequelae, such as hemiparesis and even death from intracerebral edema and brain herniation in rare instances.

Race

Almost 80% of the recorded cases include whites, with the remainder being Japanese, African American, Nepalese, and from the Indian subcontinent. Whether this racial distribution represents a predilection of APMPPE for whites or a reporting bias is unclear.

Sex

Earlier reports of APMPPE suggested a slight preponderance of women with this disease, but more recent publications suggest no sexual predilection, with equal frequency between both men and women.

Age

The mean age of onset is approximately 27 years. The documented age range of onset is 7-66 years. The most frequent age range of occurrence of APMPPE is in those patients aged 16-40 years, representing approximately 85% of cases. About 50% of patients present in the third decade of life.

Previous
Proceed to Clinical Presentation
 
 
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.

Specialty Editor Board

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.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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.

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.

Acknowledgments

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous coauthor, James P Ganley, MD, PharmD, DrPH, to the development and writing of this article.

References

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

  2. Yang DS, Hilford DJ, Conrad D. Acute posterior multifocal placoid pigment epitheliopathy after meningococcal C conjugate vaccine. Clin Experiment Ophthalmol. Apr 2005;33(2):219-21. [Medline].

  3. Mendrinos E, Baglivo E. Acute posterior multifocal placoid pigment epitheliopathy following influenza vaccination. Eye (Lond). Jan 2010;24(1):180-1. [Medline].

  4. Fine HF, Kim E, Flynn TE, Gomes NL, Chang S. Acute posterior multifocal placoid pigment epitheliopathy following varicella vaccination. Br J Ophthalmol. Mar 2010;94(3):282-3, 363. [Medline].

  5. Yeh S, Forooghian F, Wong WT, Faia LJ, Cukras C, Lew JC, et al. Fundus autofluorescence imaging of the white dot syndromes. Arch Ophthalmol. Jan 2010;128(1):46-56. [Medline]. [Full Text].

  6. Cheung CM, Yeo IY, Koh A. Photoreceptor changes in acute and resolved acute posterior multifocal placoid pigment epitheliopathy documented by spectral-domain optical coherence tomography. Arch Ophthalmol. May 2010;128(5):644-6. [Medline].

  7. 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].

  8. 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].

  9. 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].

  10. 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].

  11. 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].

  12. 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].

  13. Lowder CY, Foster RE, Gordon SM, Gutman FA. Acute posterior multifocal placoid pigment epitheliopathy after acute group A streptococcal infection. Am J Ophthalmol. Jul 1996;122(1):115-7. [Medline].

  14. 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].

  15. 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].

  16. Parmeggiani F, Costagliola C, D'Angelo S, Incorvaia C, Perri P, Sebastiani A. Clear cell renal cell carcinoma associated with bilateral atypical acute posterior multifocal placoid pigment epitheliopathy. Oncology. 2004;66(6):502-9. [Medline].

  17. Ryan SJ, Maumenee AE. Acute posterior multifocal placoid pigment epitheliopathy. Am J Ophthalmol. Dec 1972;74(6):1066-74. [Medline].

  18. Scheufele TA, Witkin AJ, Schocket LS, Rogers AH, Schuman JS, Ko TH, et al. Photoreceptor atrophy in acute posterior multifocal placoid pigment epitheliopathy demonstrated by optical coherence tomography. Retina. Dec 2005;25(8):1109-12. [Medline]. [Full Text].

  19. 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].

  20. Thomson SP, Roxburgh ST. Acute posterior multifocal placoid pigment epitheliopathy associated with adenovirus infection. Eye. May 2003;17(4):542-4. [Medline].

  21. Uthman I, Najjar DM, Kanj SS, Bashshur Z. Anticardiolipin antibodies in acute multifocal posterior placoid pigment epitheliopathy. Ann Rheum Dis. Jul 2003;62(7):687-8. [Medline].

  22. 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].

 
Previous
Next
 
Posterior pole of right eye. Early acute posterior multifocal placoid pigment epitheliopathy lesion shows yellowish-white placoid lesion involving the macula and an area just inferior temporal to the macula.
Fluorescein angiography showing peripheral hypofluorescence and central leakage of the lesion inferior temporal to the macula.
Posterior pole of left eye of same patient showing acute posterior pole placoid lesion.
Fluorescein angiogram of the patient above in late phase showing late staining of placoid areas.
Inferior nasal of right eye of the same patient approximately 2 months later, showing scattered areas of retinal pigment epithelium atrophy and hyperplasia.
Fluorescein angiography of same patient in late phase showing areas of late staining.
This image shows localized scleritis superiorly.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.