Familial band-shaped nodular keratopathy

A family is reported in which two brothers have a bilateral, symmetrical corneal disorder characterized by small yellowish to amber-colored globules arranged in a band-shaped configuration in the central superficial layers of the cornea. The globules have a similar clinical appearance to those found in Bietti's "band-shaped nodular dystrophy," a keratopathy that is nonfamilial, has an onset later in life, and initially involves the peripheral cornea. Light and electron microscopic studies of corneal tissue obtained from the proband showed that the globular deposits autofluoresce, that they stain black with Verhoeff's elastic stain, and that they correspond to an electron-dense material found within corneal epithelial cells and keratocytes and within the extracellular tissues of the anterior cornea. A third, younger brother appears to have the early corneal signs of the condition. Unlike the condition described by Bietti, the keratopathy we report is unique in its familial nature, early onset in life, and initial, marked central corneal involvement.