- Autosomal dominantly inherited.
- Glare, foreign body sensation, decreased vision or recurrent painful epithelial erosions
- In young adults, vision is usually unaffected
- By the age 40, visual acuity will decrease insidiously as the lesions increase in size, multiply, coalesce, and distribute to a deeper stroma
- Relatively symmetric, discrete, well demarcated crumb-like, white-grey granules in the axial anterior central stroma sparing the 1-3 mm of periphery cornea
- Stroma in between lesions remains clear
- Usually becomes apparent with recurrent corneal erosions during the first and second decade of life
- Superficial debridement, lamellar keratoplasty, and excimer laser phototherapeutic keratectomy usually provide good outcomes.
- Penetrating keratoplasty is rarely required but indicated in advanced cases with extensive stromal involvement and significant decrease in visual acuity.
- Recurrent granular dystrophy may occur after keratoplasty.