Last updated:
April 25, 2008
Years published: 1989, 1997, 2003
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.
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.
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.
Acute posterior multifocal placoid pigment epitheliopathy is a rare visual disorder that affects males and females in equal numbers.
Treatment of acute posterior multifocal placoid pigment epitheliopathy is symptomatic and supportive. Very often vision returns without specific treatment.
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For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
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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.
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The information provided on this page is for informational purposes only. The National Organization for Rare Disorders (NORD) does not endorse the information presented. The content has been gathered in partnership with the MONDO Disease Ontology. Please consult with a healthcare professional for medical advice and treatment.
The Genetic and Rare Diseases Information Center (GARD) has information and resources for patients, caregivers, and families that may be helpful before and after diagnosis of this condition. GARD is a program of the National Center for Advancing Translational Sciences (NCATS), part of the National Institutes of Health (NIH).
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