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- Rare, autrosomal dominantly inherited.
- Often asymptomatic with no visual significant
- May be associated with mild photophobia
- Corneal erosions have been reported
- 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.