Acute Angioid Streak Retinopathy is a Two-Phase Inflammatory Process Starting with Outer Retinal Inflammation, Followed by Secondary MEWDS.

Purpose: To present a case of recurrent acute angioid streak retinopathy (AASR) and discuss its potential pathophysiological mechanisms based on multimodal imaging findings.

Observations: A 19-year-old woman with pseudoxanthoma elasticum (PXE) experienced three distinct episodes of posterior segment inflammation associated with angioid streaks (AS), previously identified as AASR. Each episode started with acute subretinal inflammation due to a disruption of the retinal pigment epithelium (RPE)/Bruch's membrane (BM) complex at an AS site, which appeared as a peri-AS whitening on fundus photography. About one week later, as the peri-AS lesions resolved, multifocal white dots characteristic of multiple evanescent white dot syndrome (MEWDS) appeared around the optic disc and at the posterior pole. Multimodal imaging, including blue autofluorescence, fluorescein angiography, indocyanine green angiography, optical coherence tomography (OCT), and OCT-angiography, confirmed these findings. While the peri-AS lesions resembled multifocal choroiditis, they differed in their extension along the AS and faster resolution. Each AASR episode resulted in a detectable AS enlargement on fundus autofluorescence. These findings suggested that AASR could involve a two-phase inflammatory process: an initial disruption of the RPE/BM complex at an AS site triggering localized choroiditis/outer retinitis, followed by secondary MEWDS and subsequent AS enlargement.

Conclusion: This case illustrates a recurring inflammatory pattern in PXE-associated AS, characterized by localized inflammation at AS sites with RPE/BM disruption, and secondary MEWDS. These findings highlight the pathophysiological mechanisms of AASR and its unique imaging features.