Central cloudy corneal dystrophy of François

A family is reported with central cloudy dystrophy of the cornea, as described by François in 1956. The lesion consists of a grey zone deep in the stroma, made up of polygonal or rounded areas with indistinct margins, separated by apparently clear, normal stroma. No deposits are visible in the slit lamp. The anterior and posterior boundary layers appear normal. The corneal thickness is normal. Open angle glaucoma was present in the family. The pathogenesis of the dystrophy is unknown, and histopathological examination has not been described. The differential diagnosis is briefly discussed. The family tree is suggestive of a dominant mode of transmission.