Superficial reticular degeneration of Koby

A 36-year-old white man with congenital ichthyosis and a history of a posterior uveitis beginning at age 8 presented with an unusual corneal opacity. At age 26 bilateral central corneal opacities were noted consisting of fine interlacing white lines forming a reticulum at the level of Bowman's membrane. A faint brown background tint of the basal epithelium was also present. Over the next 10 years the opacity progressed only slightly, with some mild superficial scarring of the underlying corneal stroma. The visual acuity remained good. This corneal pattern appeared virtually identical to the superficial reticular degeneration of Koby. As described by Koby in 1927, this rare corneal degeneration is characterised as a painless, slow, progressive development of a central fine, white reticulum at the level of Bowman's membrane. This rare corneal degeneration probably represents an atypical form of band keratopathy.