Corneal Dystrophy, Lisch Epithelial

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.