Junctional Epidermolysis Bullosa

Abstract

• Junctional epidermolysis bullosa (EB) is a rare, heritable, blistering disease of the skin characterized by presence of bullae at birth, lack of scarring of the lesions, and early death. To date there has been no effective treatment for the disease. Phenytoin, which decreases collagenase activity in human skin explants and fibroblast cultures, has been used successfully to treat patients with recessive dystrophic EB. We found a marked decrease in new blister formation in one child with junctional EB during phenytoin therapy. (Am J Dis Child 1983;137:992-994)