Of the anterior epithelial dystrophies, this one is unusual in the absence of spontaneous corneal erosions. It is also the only one of this group to be an X-linked disorder. Onset occurs in childhood and by adulthood vision can be significantly reduced. Most individuals, however, retain vision in the range of 20/25 to 20/40 into the 7th and 8th decades. Gray, feathery opacities in the anterior cornea often appear in a band-like or whorled pattern. Vacuolization of the epithelial cells is responsible for this appearance but in Lisch dystrophy, these microcysts are empty compared to Reis-Bucklers, epithelial basement membrane, and Thiel-Behnke anterior dystrophies in which degenerative debris fills them. In spite of the X-linked inheritance pattern, both males and carrier females may have similar corneal opacities since the mutation is dominant.
