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Angioid Streaks and Pseudoxanthoma Elasticum

Paul N. Schultz, MD; Warren M. Sobol, MD
JAMA. 1991;265(1):45. doi:10.1001/jama.1991.03460010045028.
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Angioid streaks are irregular, peripapillary brown to red curvilinear bands that extend in a weblike manner from the optic disc (Fig 1). Often confused with blood vessels, angioid streaks are wider, with tapering ends, lie below the normal retinal vasculature, and are readily discerned by direct ophthalmoscopy. Their importance lies in their association with a host of systemic disorders.

Pseudoxanthoma elasticum (PXE), Paget's disease of the bone, and sickle-cell disease account for the majority of diseases linked to angioid streaks. PXE can be demonstrated in more than half the patients with angioid streaks1,2; in one large series, 85% of patients with PXE demonstrated angioid streaks.3 Additional associated conditions include acromegaly, abetalipoproteinemia, diabetes mellitus, Ehlers-Danlos syndrome, hemochromatosis, facial hemochromatosis, hemolytic anemia, hypercalcinosis, hyperphosphatemia, neurofibromatosis, senile elastosis, Sturge-Weber syndrome, tuberous sclerosis, lead poisoning, and myopia.1

Additional ocular findings in PXE include a diffuse, yellowish mottling of the retinal pigment


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