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Acute posterior multifocal placoid pigment epitheliopathy


Other Names:
Epitheliopathy, acute posterior multifocal placoid pigment; Acute multifocal placoid pigment epitheliopathy; Acute placoid pigment epitheliopathy; Epitheliopathy, acute posterior multifocal placoid pigment; Acute multifocal placoid pigment epitheliopathy; Acute placoid pigment epitheliopathy; AMPPE; APMPPE; Multifocal placoid pigment epitheliopathy See More
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Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an acquired, inflammatory eye condition affecting the retina, retinal pigment epithelium (pigmented layer of the retina), and choroid. It usually affects both eyes and is characterized by multiple, yellow-white lesions in the back of the eye. The condition can significantly impair visual acuity if the macula is involved.[1] APMPPE typically resolves on its own in weeks to months. While the cause is unknown, about a third of cases appear to develop after a flu-like illness.[2] Non-ocular symptoms are uncommon, but cerebral vasculitis can be present and may cause permanent and/or severe neurological complications.[1]
Last updated: 9/9/2014
Prior to onset of the condition, about a third of people have flu-like or viral symptoms such as fever, swollen lymph glands, nausea, vomiting, joint pain and/or tenderness. Headaches may also be present. Rarely, there may be neurological signs such as temporary loss of speech (aphasia) and/or weakness of the arms and legs.[1][3]

In the early stages of APMPPE, affected people may notice areas of visual blotchiness; flashes of light (photopsia) caused by irritation of the retina; distortion of shapes (metamorphopsia); increased sensitivity to light (photophobia); and/or conjunctivitis. Later, affected people usually develop impairment of vision. In rare cases, vision impairment may be severe.[1][3]

In most cases, the disorder resolves within a few weeks without permanent loss of visual acuity. However, in some cases, visual acuity does not improve.[3] Neurological symptoms develop in some affected people and should be evaluated and treated promptly, as neurological involvement can result in severe complications.[4][1]

There are rare cases of chronic or recurrent APMPPE; it has been suggested that these cases represent relentless placoid chorioretinitis.[5] This is a rare condition characterized by the development of multiple inflammatory lesions resembling those seen in APMPPE. However, unlike in APMPPE, the lesions continue to expand in size and number, with a relentless course over many months.[6]
Last updated: 9/9/2014
The treatment of APMPPE is somewhat controversial, but the general consensus is that no treatment seems to alter the course of the ocular lesions. In cases complicated by subretinal neovascularization (growth of new blood vessels), laser photocoagulation may be useful.[1]

In most cases, the lesions resolve spontaneously and no therapy is required. Some clinicians have used corticosteroids (which suppress inflammation) to treat the ocular findings and any severe systemic involvement. However, there is no evidence that treatment with corticosteroids affects the visual outcome.[1] The use of steroids has also been suggested when treating cases where the macula is involved.[7] Cycloplegics may be useful for severe iritis, which is an uncommon finding.[1]

It is recommended that people with questions about treatment options for themselves or family members speak with their health care provider.
Last updated: 9/9/2014
In many affected people, visual acuity returns to the range of 20/20 to 20/40. However, some people have permanent vision loss or long-term functional ocular symptoms. Other ocular and non-ocular symptoms usually resolve on their own and are not life-threatening. In affected people with cerebral vasculitis, there can be permanent and/or severe neurological complications; there have been instances of death following an episode of cerebral vasculitis.[1]
Last updated: 9/9/2014

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Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The American Academy of Ophthalmology Web site has an information page on Acute posterior multifocal placoid pigment epitheliopathy. Their Web site is dedicated to educating people about eye diseases and conditions and the preservation of eye health.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Acute posterior multifocal placoid pigment epitheliopathy. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • I have the version of AMPPE that does not go away in a few months. I've had it since 2010. My vision seems to be getting darker, even in a brightly lit room -- the longer I read, the darker it gets. I have deposits on the macula of both eyes. Has anyone ever noted increasing memory problems after the AMPPE starts? I'm wondering if there are deposits in the brain similar to the deposits on the eyes, but with the deposits in the brain causing memory loss. Has anyone tried removing the deposits on the macula, perhaps with a laser? Do the deposits damage the macula? See answer

  • My husband has been diagnosed with acute posterior multifocal placoid pigment epitheliopathy. Is the vision loss associated with this condition permanent? See answer


  1. Lakshmana M Kooragayala. Acute Multifocal Placoid Pigment Epitheliopathy. Medscape Reference. September 16, 2013; http://emedicine.medscape.com/article/1225531-overview. Accessed 9/9/2014.
  2. Epitheliopathy, Acute Posterior Multifocal Placoid Pigment. National Organization for Rare Disorders (NORD). 2003; https://rarediseases.org/rare-diseases/epitheliopathy-acute-posterior-multifocal-placoid-pigment/.
  3. EPITHELIOPATHY, ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT. NORD. April 25, 2008; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/609/viewAbstract. Accessed 9/9/2014.
  4. O'Halloran HS 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):861-868. Accessed 9/9/2014.
  5. Ramana S. Moorthy. Recognizing the ‘White Dot’ Syndromes. Review of Ophthalmology. June 11, 2009; http://www.revophth.com/content/d/retinal_insider/i/1215/c/22884/. Accessed 9/9/2014.
  6. Lawrence A. Yannuzzi. Relentless Placoid Chorioretinitis (Ampiginous Choroiditis). The Retinal Atlas. Elsevier; 2010;
  7. Grkovic D, Oros A, Bedov T, Karadžic J, Gvozdenovic L, Jovanovic S. Acute posterior multifocal placoid pigment epitheliopathy-retinal "white dot syndrome". Med Glas (Zenica). February, 2013; 10(1):194-196. Accessed 9/9/2014.