Acute Posterior Multifocal Placoid Pigment Epitheliopathy Spectrum on Multimodal Imaging: A Severity-Driven Ischemic Continuum

PURPOSE: To define multimodal imaging-based patterns across the Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) spectrum and relate imaging features to their pathogenesis. METHODS: This retrospective, observational, single-center study included consecutive patients with APMPPE-spectrum disease. At the first evaluation, eyes were categorized in three patterns based on color fundus photography (CFP), fundus autofluorescence (FAF) and indocyanine green angiography (ICGA): (i) classic APMPPE with placoid lesions only at posterior pole (PP); (ii) peripheral APMPPE with placoid lesions involving only PP on FAF, but extending to the mid-periphery (MP) and far periphery (FP) on CFP and ICGA; (iii) ampiginous choroiditis (AC) with mixed placoid/serpiginoid lesions from PP to FP. RESULTS: Overall, 19 eyes of 10 patients (8 males, 2 females; median age 23 years [IQR 21-31]) were included: of these, 2 patients (3 eyes) were categorized in classic APMPPE, 4 patients (8 eyes) in peripheral APMPPE, and 4 patients (8 eyes) in AC. At the first examination, a hyperfluorescent peripheral vessel abutting plaques on late ICGA was identified in 9/19 eyes (APMPPE 3/3; peripheral APMPPE 3/8; AC 3/6). At the last examination, FP FAF hypoautofluorescence was present in 100% of eyes with AC (8/8) and absent in all eyes with classic and peripheral APMPPE; MP hypoautofluorescence paralleled this gradient; PP hypoautofluorescence occurred in all patterns. CONCLUSIONS: Using multimodal imaging approach, we describe a distinct phenotypic pattern termed peripheral APMPPE (PAPMPPE) that exhibits an intermediate phenotype between classic APMPPE and AC, supporting the concept of a severity-graded continuum.