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Tuesday, August 28, 2007

Fabry's disease




The most common and first ocular sign of Fabry’s disease: bilateral corneal diffuse yellow epithelial haziness that gradually becomes concentrated into dense rays that radiate from the center of the cornea into dense bronze to cream-colored streaks arranged in a vortex or star-shaped pattern (whorl-like or verticillata).8,9 These opacities appear to be in the subepithelial or Bowman’s layer of the cornea.8 Corneal involvement occurs in over 90% of these patients,