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December 13, 2018

Angioid Streaks Are Not a Common Feature of Ehlers-Danlos Syndrome

Author Affiliations
  • 1Wilmer General Eye Services, Baltimore, Maryland
  • 2Wilmer Eye Institute, Baltimore, Maryland
JAMA Ophthalmol. 2019;137(3):239. doi:10.1001/jamaophthalmol.2018.5995

A tenet of ophthalmology is that angioid streaks (AS) are associated with Ehlers-Danlos syndrome (EDS). Notably, AS have been associated with other conditions, such as pseudoxanthoma elasticum, Paget disease of bone, and sickle cell disease, and they can also be seen idiopathically. Indeed, most ophthalmologists in training are taught the mnemonic “PEPSI” to help remember these associations.1 A review of the published literature suggests that all articles and reviews that mention the association of AS and EDS ultimately refer to a publication by Green et al.2 This report mentions that 2 members from a family with EDS, a mother and daughter, demonstrated AS in their retinas. Notably, the authors described findings for 15 members of 2 families with EDS and none of the other patients demonstrated AS. The authors went on to mention that theirs was the first report describing the presence of AS in EDS.

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