Multimodal imaging of angioid streaks in pseudoxanthoma Elasticum

A 33-year-old woman with pseudoxanthoma elasticum confirmed by dermatologic examination, elastorrhexis on biopsy, and ABCC6 mutations underwent multimodal imaging. Best-corrected visual acuity was 20/20 bilaterally. Color fundus photography (Figure 1A) revealed the opaque appearance of calcified Bruch's membrane with a peau d’orange transition zone and multiple angioid streaks radiating from the disc, ¹ corresponding to breaks in brittle, calcified Bruch's membrane. Fundus autofluorescence (Figure 1B) showed hypoautofluorescent bands matching some visible streaks, reflecting RPE alteration. Spectral-domain OCT (Figure 1C) demonstrated focal Bruch's membrane discontinuities with subtle ellipsoid zone and RPE disruption. ² Adaptive optics (Figure 1D) highlighted microstructural remodeling within a type 2 streak. ³

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Figure 1.

(A) Color fundus photograph of the left eye showing angioid streaks radiating from the optic disc, the opaque appearance of calcified Bruch’s membrane in the posterior pole, and the characteristic peau d’orange transition zone in the mid-temporal retina (white arrows). (B) Fundus autofluorescence shows radial hypoautofluorescent bands corresponding to some clinically visible streaks, reflecting retinal pigment epithelium alteration. (C) Optical coherence tomography through a streak demonstrates focal discontinuity of Bruch's membrane (red arrow) with subtle ellipsoid zone irregularities. (D) Adaptive optics imaging reveals a hyporeflective streak with internal hyperreflective foci, illustrating localized microstructural remodeling of the RPE–Bruch's membrane complex.