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Pseudoxanthoma elasticum (PXE) is a rare autosomal recessive disease of the connective tissue which primarily affects the skin, the retina and the cardiovascular system.1 PXE is characterised by mineralisation and fragmentation of elastic fibres. Eye involvement results from calcification and breaks in the Bruch’s membrane (BM), causing a variety of ocular fundus changes (figure 1).1 ,2 Among them, angioid streaks can be complicated by choroidal neovascularisation and/or macular atrophy resulting in central visual loss.1 Although central visual loss in PXE profoundly impacts quality of life,1 little is known about other risk factors for visual impairment. The aim of our study was to determine if visual impairment related to maculopathy in PXE may be associated with age and other specific retinal features.
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