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Invited Commentary
August 8, 2019

Potentially Novel Acute Retinopathy Similar to Multiple Evanescent White Dot Syndrome in Patients With Pseudoxanthoma Elasticum

Author Affiliations
  • 1Department of Ophthalmology, Tufts Medical Center, Boston, Massachusetts
JAMA Ophthalmol. Published online August 8, 2019. doi:10.1001/jamaophthalmol.2019.2884

In this issue of JAMA Ophthalmology, Gliem et al1 present a case series describing a potentially novel presentation of acute retinopathy in 9 patients with pseudoxanthoma elasticum (PXE). Clinical findings in this disease shared many characteristics with multiple evanescent white dot syndrome (MEWDS): female predominance (8 of 9 patients), unilaterality (9 of 9 patients), blurry visual acuity and/or photopsia (variable symptoms among the cohort), mild vitreous cells (6 of 9 patients), and multiple small punctate outer retinal white lesions that tended to cluster in the posterior pole, particularly around the optic nerve and retinal vessels. Ancillary testing findings that compared similarly with MEWDS included reduced electroretinogram amplitudes in some patients (3 of 6), many more apparent lesions on fundus autofluorescence imaging and indocyanine green angiography, with hypocyanescence of the lesions on indocyanine green angiography, hyperfluorescence of lesions on fluorescein angiography, and outer retinal disruptions on optical coherence tomography.2