Atypical corneal dystrophy with stromal amyloid deposits

Corneal dystrophies are distinctive clinically and histopathologically; however, variations do occur. We present two cases of stromal amyloidosis from one family masquerading other corneal dystrophy. The two cases are from a six-generation family with an autosomal dominant corneal dystrophy resembling Reis-Bucklers' dystrophy. In these cases, neither the propositus nor other family members showed typical lattice lines. Light and electron microscopy of the obtained corneal buttons disclosed amyloid stromal deposits. The clinicopathologic correlation of these cases suggests that this family represents a variant of stromal amyloid dystrophies.