EPIDERMOLYSIS-BULLOSA SIMPLEX ASSOCIATED WITH MUSCULAR-DYSTROPHY - A NEW CASE

We report an infant with a rare form of epidermolysis bullosa simplex characterized by an autosomal recessive pattern of inheritance, severe cutaneous involvement, oral and nail lesions, association with muscul ar dystrophy, and a poor prognosis, due to extracutaneous disease. In addition to the usual presentation of this disease, our patient had se vere anemia, with immature circulating white cells, and bone marrow hi stology suggestive of a pre-leukemic state, a finding which has not be fore been reported in the literature.