- A slowly progressive, uncommon, dominantly inherited condition.
- Usually bilateral but often asymmetric.
- Variable clinical features include isolated or coalescent posterior corneal vesicular (the most distinctive characteristic), multi-layered Descemet's membrane thickening and band-like configuration with sharp scalloped margin.
- The peripheral abnormal multi-layered endothelial cells may cause localized iridocorneal adhesion.
- Symptom: most cases are asymptomatic and do not require treatment.
- If the condition progresses to corneal decompensation, definitive treatment with penetrating keratoplasty is usually required.