Corneal involvement in epidermolysis bullosa simplex
H. Granek and H. P. Baden
A 17-year-old boy and his mother represent the first reported cases of
ocular (corneal) involvement of the simplex form of epidermolysis bullosa.
Both had a ring-like configuration of fine bullous lesions in the
midperiphery bilaterally at the level of deep corneal epithelium
superficial to Bowman's membrane (basal cell layer), with the son
manifesting symptoms when some of his bullae ruptured through to the
corneal epithelial surface. Nonscarring blistering was present in three
generations of this family, suggestive of dominantly inherited
epidermolysis bullosa simplex, and was confirmed by electron microscopy of
a skin specimen from the son. Thus, ocular involvement has now been
observed in all of the major types of epidermolysis bullosa.
