Pseudoxanthoma elasticum patients with angioid streaks are well-known to have acute vision loss due to choroidal bleeding.
However, chronic vision loss due to macular atrophy is less well characterized. We describe a patient with sub-acute vision
loss in one eye due to loss of macular retinal pigment epithelium function. Autofluorescence and pattern electroretinogram
were useful adjuncts to help diagnose the source of her vision loss.
Keywords Pseudoxanthoma elasticum - Autofluorescence - Pattern electroretinogram - Maculopathy - Angioid streaks