unknown

This disorder, reported so far in a single family, is an anterior corneal dystrophy with onset in the first decade of life.  The frequency of epithelial erosions tended to subside during adolescence but visual acuity continued to decline secondary to subepithelial nodular opacities and a generalized haze most dense centrally. No geographic lines are present and cystic changes in the epithelium were absent.  Bowman layer and deeper stuctures of the cornea are unaffected. Patients may have 20/30 vision into the fifth decade but after that it may decrease into the 20/400 range.  EM revealed accumulations of subepithelial fibrillar material.  Light microscopy and immunohistochemistry showed the material to be chondroitin-4-sulfate and dermatan sulfate.