- Autosomal dominantly inherited with very high penetrance and moderately variable expressivity.
- Symptoms: glare, foreign body sensation, decreased vision or recurrent painful epithelial erosions.
- Bilateral, often asymmetric, grey-white well-circumscribed central anterior stromal opacity similar to those seen in granular dystrophy
- Deep lattice-like stromal deposits
- Older patients have anterior stromal haze between deposits
- Family trace study revealed that patients with this dystrophy were from Avellino, Italy
- Superficial debridement, lamellar keratoplasty, and excimer laser phototherapeutic keratectomy.
- Penetrating keratoplasty is indicated for deeper involvement.
- Recurrence may occur after keratoplasty.