Angioid Streaks and Pseudoxanthoma Elasticum

Pseudoxanthoma elasticum (PXE) with the associated eye lesions called angioid streaks (AS) is a rare disease that can lead to severe vision loss. PXE affects several organs: the eyes, the skin, the cardiovascular system to name but a few and it manifests itself at the eye level by the appearance of choroidal-pigmented epithelium lesions called angioid streaks.

Angioid streaks can exist also in isolation, without being associated with PXE or other pathologies.

Angioid streaks appear in the form of orange-pigmented retinal lesions and they are the result of localized breaks in Bruch's membrane (an anatomic layer between the retina and the choroid).

Angioid streaks are then zones of weakness that can facilitate the appearance of choroidal neovessels (CNV) with resultant drop in visual acuity.

The appearance of CNV in patients having AS is an extremely common complication (from 72% up to 86% of eyes). Without treatment the natural history is that of a severe drop in visual acuity, often lower than 1/10 and sometimes as low as  legal blindness. It affects most often young patients and has a heavy socio-economic impact with a dramatic family repercussion. Nowadays AS complicated by CNV can be cured by means of intravitreal therapy of anti-VEGF agents.