Epitheliopathy, Acute Posterior Multifocal Placoid Pigment

Epitheliopathy, Acute Posterior Multifocal Placoid Pigment

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Epitheliopathy, Acute Posterior Multifocal Placoid Pigment is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • APMPPE
  • AMPPE
  • Acute multifocal placoid pigment epitheliopathy
  • Acute placoid pigment epitheliopathy
  • Multifocal placoid pigment epitheliopathy

Disorder Subdivisions

  • None

General Discussion

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare eye disorder of unknown (idiopathic) cause. The disorder is characterized by the impairment of central vision in one eye (unilateral) but, within a few days, the second eye may also become affected (bilateral). In most cases, the disorder resolves within a few weeks without loss of clearness of vision (acuity). However, in some cases, visual acuity does not improve. This disorder occurs predominantly in young adults, with a mean age of onset of 27 years. It is reported that, in approximately one-third of the cases, an influenza-like illness preceded the development of the disorder.

Symptoms

Prior to the onset of disease, about 33% of patients present with flu-like or viral symptoms such as fever, swollen lymph glands, nausea, vomiting, joint pain and/or tenderness. Moderate to severe headaches may also be present and, much more rarely, there may be neurological signs such as temporary loss of speech (aphasia) and/or weakness of the arms and legs.



In the early stages of APMPPE, patients notice areas of visual blotchiness within the field of clear vision (blotchy scotomata), flashes of light (photopsia) caused by irritation of the retina, distortion of the shapes of objects (metamorphopsia), increased sensitivity to light (photophobia) and conjunctivitis.



During the late stages of the disorder, patients usually notice mild decreases in vision. Rarely, the impaired vision may be severe.



Examination of the eye usually shows multiple flat, yellow-white lesions of the posterior pole of the nerve-rich membrane lining the eyes (retina). Frequently the veins of the retina are inflamed (vasculitis), but the inflammation often subsides without treatment. In some cases, pigment changes in the retina may be permanent and the resulting visual impairment may also be permanent. However, in most cases the disorder is characterized by a temporary impairment of vision.

Causes

The exact cause of acute posterior multifocal placoid pigment epitheliopathy is not known. Researchers suspect that it may be caused by a virus. It can subside without treatment or it may recur at any time. The viruses may stay dormant in humans for extended periods of time, then for reasons yet unknown may unexplainably become reactivated.

Affected Populations

Acute posterior multifocal placoid pigment epitheliopathy is a rare visual disorder that affects males and females in equal numbers.

Standard Therapies

Treatment of acute posterior multifocal placoid pigment epitheliopathy is symptomatic and supportive. Very often vision returns without specific treatment.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOK

Kanski JJ, ed. Clinical Ophthalmology. 4th ed. Butterworth-Heinemann. Oxford, UK; 1999:170.



REVIEW ARTICLES

Stanga PE, Lim JI, Hamilton P. Indocyanine green angiography in chorioretinal diseases: an evidence-based update. Opthalmology. 2003;81:15-21.



Daniele S, Daniele C, Orcidi F, et al. Progression of choroidal atrophy in acute posterior multifocal placoid pigment epitheliopathy. Ophthalmologica. 1998;212:66-72.



JOURNAL ARTICLES

Uthman I, Najjar DM, Kanj SS, et al. Anticardiolipin antibodies in acute multifocal posterior placoid pigment epitheliopathy. Ann Rheum Dis. 2003;62:687-88.



Thomson SP, Roxburgh ST. Acute posterior multifocal placoid pigment epitheliopathy associated with adenovirus infection. Eye. 2003;17:542-44.



Hsu CT, Harlan JB, Goldberg MF, et al. Acute posterior multifocal placoid pigment epitheliopathy associated with a systemic necrotizing vasculitis. Retina. 2003;23:64-68.



Muirhead PG. Acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Clin Exp Optom. 1998;81:203-04.



Di Crecchio L, Parodi MB, Saviano S, et al. Acute posterior multifocal placoid pigment epitheliopathy and ulcerative colitis: a possible association. Acta Ophthalmol Scand. 2001;79:319-21.



Cimino L, Auer C, Herbort CP. Sensitivity of indocyanine green angiography for the follow-up of active inflammatory chorio-capillaropathies. Ocul Immunol Inflamm. 2000;8:275-83.



De Souza S, Aslanides IM, Altomare F. Acute posterior multifocal placoid pigment epitheliopathy associated with retinal vasculitis, neovacularization and subhyaloid hemorrhage. Can J Ophthalmol. 1999;34:343-45.



FROM THE INTERNET

Ganley JP, Kooragayala LM. Acute multifocal placoid pigment epithelio-pathy. EMedicine. Last Updated: August 22, 2001. 14pp.

Resources

Lighthouse International

111 E 59th St

New York, NY 10022-1202

Tel: (800)829-0500

Email: info@lighthouse.org

Internet: http://www.lighthouse.org



NIH/National Eye Institute

31 Center Dr

MSC 2510

Bethesda, MD 20892-2510

United States

Tel: (301)496-5248

Fax: (301)402-1065

Email: 2020@nei.nih.gov

Internet: http://www.nei.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

This information does not replace the advice of a doctor. Healthwise, Incorporated disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the Terms of Use . How this information was developed to help you make better health decisions.

Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.