Peripheral Center Sparing Presentation of Schnyder Corneal Dystrophy

A 67-year-old White man was referred for corneal opacities observed during routine examination. He was asymptomatic with unremarkable medical and family history. Best-corrected photopic visual acuity was 20/20 bilaterally. Slit-lamp examination of both eyes showed arcuate paracentral subepithelial crystals extending centripetally from the midperipheral cornea overlying an arc of dense paracentral haze with well-demarcated margins sparing the visual axis (A and B). Arcus lipoides was also noted. Anterior segment-OCT demonstrated focal subepithelial hyperreflectivity on both sides of the line scans (C and D). Subepithelial crystalline deposits were observed by in vivo confocal microscopy (E and F). Polymerase chain reaction identified heterozygous N102S mutation in the UBIAD1 gene, confirming the clinical diagnosis of Schnyder corneal dystrophy