Bilateral anterior ischemic optic neuropathy, pseudoxanthoma elasticum and platelet hyperaggregability

Pseudoxanthoma elasticum (PXE) is a systemic disease that affects the elastic fibers of the arterial walls producing dilatation, occlusion and eventually rupture of arteries. The lowering of visual acuity in patients harboring PXE is currently thought to account for the presence of fibrovascular ingrowth into the angioid streaks. The authors saw a 46-year-old patient with bilateral lowering of visual acuity due to bilateral anterior ischemic optic neuropathy (AION) but in whom uncomplicated angioid streaks, PXE and an increased platelet agglutination were found.