Slit lamp view demonstrates subtle grayish specks throughout the stroma.
  • Rare, autrosomal dominantly inherited.

Clinical Features

  • Symptoms:
    • Often asymptomatic with no visual significant
    • May be associated with mild photophobia
    • Corneal erosions have been reported
  • Signs:
    • Slowly progressive, diffuse, bilateral, irregularly shaped opacities in the posterior stroma of the cornea
    • The stromas in between lesions are typically clear
  • Histopathology: abnormal keratocyte swelling with membrane-limited intracytoplasmic vacuoles containing complex lipids and glycosaminoglycans
  • Treatment is not required.