Case 14 - APMPPE

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is a rare inflammatory eye condition that primarily affects the retina, particularly the pigment epithelium. It is characterized by the sudden onset of multiple yellow-white lesions in the posterior pole of the eye, typically bilateral but not necessarily symmetric.
Here are key features and characteristics of APMPPE:
1. *Onset and Presentation:*
- *Acute Onset:* APMPPE typically presents acutely with sudden vision changes.
- *Bilateral Involvement:* While it primarily affects both eyes, the severity can vary between the eyes.
- *Posterior Pole Involvement:* Lesions are often found in the posterior pole of the eye, which includes the macula.
2. *Clinical Features:*
- *Yellow-White Lesions:* The hallmark of APMPPE is the presence of multiple, discrete yellow-white placoid lesions in the posterior pole.
- *Fundus Changes:* These lesions can be observed during a dilated fundus examination. They may appear at the level of the retinal pigment epithelium (RPE) and outer retina.
3. *Visual Symptoms:*
- *Visual Disturbances:* Patients may experience visual disturbances, including blurred vision or scotomas (blind spots).
- *Central Vision Affected:* Since the macula is often involved, central vision can be significantly affected.
4. *Systemic Associations:*
- *Flulike Symptoms:* In some cases, patients may experience flu-like symptoms before the onset of visual symptoms.
- *Systemic Manifestations:* While primarily an ocular condition, APMPPE has been associated with systemic manifestations, such as central nervous system (CNS) involvement or involvement of other organs.
5. *Diagnostic Evaluation:*
- *Fluorescein Angiography:* This imaging technique is often used to visualize the pattern and extent of the lesions and assess their impact on retinal circulation.
- *Optical Coherence Tomography (OCT):* OCT provides detailed cross-sectional images of the retina and helps evaluate the extent of involvement and potential impact on retinal layers.
6. *Course and Prognosis:*
- *Self-Limited:* APMPPE is generally a self-limited condition, and visual symptoms often improve spontaneously over time.
- *Residual Changes:* Some patients may experience residual changes, and visual recovery may vary.
7. *Management:*
- *Observation:* In many cases, APMPPE is managed by observation, as the condition tends to resolve on its own.
- *Corticosteroids:* In severe cases or when there are systemic manifestations, corticosteroids may be considered to reduce inflammation.
8. *Follow-up:*
- *Regular Monitoring:* Patients are typically monitored regularly to assess the resolution of lesions and any potential complications.
While APMPPE primarily affects the eyes, it is important to consider potential systemic associations. The condition is relatively rare, and the exact cause remains unclear. Diagnosis and management are typically handled by ophthalmologists with expertise in inflammatory eye conditions.

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