Familial corneal scarring: a new dystrophy?

A 34-year-old white man with skin manifestations of the epidermal nevus syndrome was found to have anesthetic corneas, dry eyes, and localized opaque nodular elevations of both corneas. Similar corneal lesions, unassociated with corneal anesthesia, were found in four family members. Histology of corneal biopsies from the proband revealed superficial stromal scarring. The possible relationship between this familial condition and Salzmann's nodular corneal dystrophy, Goldenhar syndrome, and epidermal nevus syndrome is discussed.