What are the other Names for this Condition? (Also known as/Synonyms)

• Acute Multifocal Placoid Pigment Epitheliopathy (AMPPE)
• APMPPE (Acute Posterior Multifocal Placoid Pigment Epitheliopathy)
• Multifocal Placoid Pigment Epitheliopathy

What is Acute Posterior Multifocal Placoid Pigment Epitheliopathy? (Definition/Background Information)

• Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is a rare disorder affecting the choroid and retina, typically of both the eyes. It belongs to a group of disorders known as white dot syndromes
• The condition results in signs and symptoms that include vision abnormalities, blurred vision, and light-sensitivity. Acute Posterior Multifocal Placoid Pigment Epitheliopathy is generally observed in young and middle-aged adults
• The cause of Acute Posterior Multifocal Placoid Pigment Epitheliopathy is not well-known. However, it is known to be associated with certain conditions that include ulcerative colitis, sarcoidosis, and several infections
• A healthcare provider can use various eye exams, blood tests, and imaging studies to diagnose Acute Posterior Multifocal Placoid Pigment Epitheliopathy. Upon diagnosis, treatment options, such as steroids or immunomodulators, can be used to treat the condition
• The prognosis for Acute Posterior Multifocal Placoid Pigment Epitheliopathy depends on each individual's signs and symptoms, but is generally good. A majority of cases are known to get better on their own spontaneously (it is a self-limiting disorder)

Who gets Acute Posterior Multifocal Placoid Pigment Epitheliopathy? (Age and Sex Distribution)

• Acute Posterior Multifocal Placoid Pigment Epitheliopathy can present itself at any age, but is more likely to occur between the ages of 10-40 years (average age 27 years)
• Both males and females are affected
• Individuals of different racial and ethnic backgrounds can be affected

What are the Risk Factors for Acute Posterior Multifocal Placoid Pigment Epitheliopathy? (Predisposing Factors)

The risk factors for Acute Posterior Multifocal Placoid Pigment Epitheliopathy include:

• Family history of the condition
• The presence of HLA-B7 or HLA-DR2 genetic make-up indicates an increased risk

The following conditions have been associated with Acute Posterior Multifocal Placoid Pigment Epitheliopathy, although the cause-effect relationship is not definitely proven:

• Sarcoidosis
• Ulcerative colitis
• Polyarteritis nodosa
• Erythema nodosum
• Inflammation of thyroid (thyroidits)
• A variety of infections such as group A Streptococcus infection, tuberculosis, Lyme disease, adenoviruses infections, hepatitis B infections, mumps, influenza, etc.

Note: An association means that there is an increased incidence of the condition that is present with Acute Posterior Multifocal Placoid Pigment Epitheliopathy. The presence of an associated condition does not mean that it is the cause of APMPPE.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Acute Posterior Multifocal Placoid Pigment Epitheliopathy? (Etiology)

• Acute Posterior Multifocal Placoid Pigment Epitheliopathy is an eye disorder that is associated with many conditions. However, the cause is not clearly understood or identified
• Some researchers believe that it may be due to an abnormal inflammation caused by an underlying condition
• APMPPE is a type of white dot syndrome; white dot syndromes are a group of eye disorders that involve inflammation of the retina and choroid

What are the Signs and Symptoms of Acute Posterior Multifocal Placoid Pigment Epitheliopathy?

The signs and symptoms of Acute Posterior Multifocal Placoid Pigment Epitheliopathy may vary between individuals and may include:

• One-third individuals are known to have flu-like symptoms before eye-related signs and symptoms are noted
• Reduced vision, blurred vision
• Light-sensitivity or photophobia
• Flashes of light in the eye, or photopsia
• The condition is bilateral and both eyes are usually affected; one eye is affected first followed by the other
• The signs and symptoms may be asymmetrical, which means that:
  • The combination of signs and symptoms in each eye may be different
  • The rate of progression of the signs and symptoms of each eye may be different
  • The severity of signs and symptoms may vary from eye to eye

The presentations of any underlying condition may also be observed.

How is Acute Posterior Multifocal Placoid Pigment Epitheliopathy Diagnosed?

A healthcare professional may diagnose Acute Posterior Multifocal Placoid Pigment Epitheliopathy using the following tests and procedures:

• Physical examination and analysis of previous medical history
• Eye examination by an eye specialist
• Fundoscopic (ophthalmoscopic) examination by an eye specialist, who examines the back part of the eye (or the fundus)
• Visual acuity test using a special and standardized test chart (Snellen chart)
• Slit-lamp examination: Examination of the eye structure using a special instrument called a slit-lamp. In this procedure, the pupils are dilated and the internal eye structure is examined
• Tonometry: Measurement of intraocular pressure or eye fluid pressure, especially to detect conditions such as glaucoma
• Fundus fluorescein angiography (FFA): In this technique, the eye blood vessels are examined using a fluorescein dye
• Fundus autofluorescence (FAF) imaging: It is a diagnostic technique to examine the fundus of the eye using a fluorescent dye
• Indocyanine green (ICG) angiography: It is used to examine the blood vessels of the choroid using a dye, called indocyanine green, particularly to study the choroid
• B-scan ultrasonography: Special ultrasound scan of the eye through a non-invasive diagnostic tool, to assess health of the eye structures
• Electroretinogram (ERG): It is a technique to measure electrical activities in the retinal cells
• Optical coherence tomography (OCT) of eye: Radiological imaging technique to visualize the eye structure
• Blood tests that include:
  • To check for the presence of antibodies in blood
  • Blood culture for infections
  • Complete blood count (CBC) with differential
  • Erythrocyte sedimentation rate (ESR)
  • Testing to determine the presence of HLA-B7 or HLA-DR2 genetic make-up
• Other tests to determine any underlying condition causing APMPPE

If individuals have other signs and symptoms, then the following tests may be performed:

• Chest X-rays
• Neuroimaging studies including MRI scan of brain
• Lumbar puncture: In this procedure, the cerebrospinal fluid is collected and analyzed

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Acute Posterior Multifocal Placoid Pigment Epitheliopathy?

Significant complications of Acute Posterior Multifocal Placoid Pigment Epitheliopathy are generally not observed. In some cases, the complications may include:

• If left undiagnosed and/or untreated, the condition can lead to vision loss
• Kidney damage, high blood sugar, and high blood pressure due to steroid treatment
• Recurrent Acute Posterior Multifocal Placoid Pigment Epitheliopathy: The condition may recur if the underlying cause is not identified and treated appropriately. With each recurrence, the signs and symptoms may get more severe

How is Acute Posterior Multifocal Placoid Pigment Epitheliopathy Treated?

Currently, standard treatment protocols are not well-established for Acute Posterior Multifocal Placoid Pigment Epitheliopathy. However, many cases are known to resolve spontaneously and the condition is self-limiting.

The treatment may include the following measures:

• Oral medications (including pain medications)
• Steroids may be administered to control the inflammation
• Dark glasses may be prescribed for light-sensitivity
• Addressing underlying conditions that may be contributing to progression of the disorder
• If the underlying cause is unknown, then decreasing inflammation is the main step towards treating APMPPE
• Administration of immunomodulators, which are medications to control dysfunctional immune system

It is important to note that steroids may not be used in all cases, since it can worsen the condition. A healthcare provider will provide the best treatment options based upon each individual’s specific circumstances.

How can Acute Posterior Multifocal Placoid Pigment Epitheliopathy be Prevented?

It may be difficult to prevent Acute Posterior Multifocal Placoid Pigment Epitheliopathy, but the risk for the condition may be lowered through the following measures:

• Avoiding contact with individuals who have infections
• Educating the individual and family members about the underlying cause and preventing the development of any modifiable risk factors associated with the underlying condition (if possible)
• Treating any underlying infections and immunologic conditions, as early as possible
• Maintaining long-term follow-up checkups to watch out for any recurrences

What is the Prognosis of Acute Posterior Multifocal Placoid Pigment Epitheliopathy? (Outcomes/Resolutions)

• The prognosis of Acute Posterior Multifocal Placoid Pigment Epitheliopathy is generally good, as the condition is self-resolving in many cases. The signs and symptoms get better in about 4-5 weeks
• However, the prognosis also depends upon the severity of the signs and symptoms. APMPPE is known to recur and in some cases, individuals may take over 6 months to fully recover

Additional and Relevant Useful Information for Acute Posterior Multifocal Placoid Pigment Epitheliopathy:

Please visit our Eye & Vision Health Center for more physician-approved health information:

http://www.dovemed.com/health-topics/vision-center/