Unmasking Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) Through Multimodal Imaging: A Case Report

TL;DR

This case report describes a rare eye condition called APMPPE, diagnosed using advanced imaging techniques and showing spontaneous recovery without treatment.

Contribution

The paper presents a novel case of APMPPE confirmed through multimodal retinal imaging and highlights its self-limiting nature.

Findings

Multimodal imaging confirmed APMPPE features in a patient with subacute vision loss.

The patient's vision spontaneously improved over two weeks without immunosuppressive therapy.

No infectious or autoimmune causes were identified, supporting a self-limiting disease course.

Abstract

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare, immune-mediated chorioretinal inflammatory condition typically affecting healthy young adults. It often presents with acute bilateral vision loss and multiple creamy placoid lesions at the level of the retinal pigment epithelium, frequently following a flu-like or viral prodrome. We report the case of a 24-year-old male who developed bilateral subacute vision loss, three days in duration, one month after a febrile upper respiratory infection. Fundus examination revealed multiple yellow-white placoid lesions in both eyes, and multimodal retinal imaging (optical coherence tomography (OCT), fundus autofluorescence (AF), and OCT angiography (OCTA)) confirmed features consistent with APMPPE. A comprehensive infectious and autoimmune workup was negative, and neuro-imaging showed no evidence of cerebral vasculitis. The patient was managed conservatively without immunosuppressive therapy. Over the course of two weeks, his visual acuity spontaneously improved from 20/65 (logarithm of the Minimum Angle of Resolution (logMAR) = 0.5) to 20/30 (logMAR = 0.2) in the right eye and from 20/25 (logMAR = 0.1) to 20/20 (logMAR = 0) in the left eye, with corresponding regression of the retinal lesions. This case highlights the importance of recognizing APMPPE in the appropriate clinical context through non-invasive multimodal imaging, distinguishing it from infectious or chronic placoid retinopathies, emphasizing the role of careful observation and follow-up in managing this condition with a favorable visual prognosis.

Figures