Epidermolysis Bullosa Heriditaria: - a brief survey and clinical data on 56 living persons with epidermolysis bullosa dystrophica dominans

Abstract

A brief survey of the disease epidermolysis bullosa heriditaria is given. In the Faroe Islands, epidermolysis bullosa has been found to occur in two families. Among a total of 73 affected individuals, 56 are still alive. The disease was found to be systematically transmitted by a dominant, autosomal gene. According to a review of the clinical findings in the indívidual cases, the dísease was of dystrophic type, the symptoms including deformities of nails, scarring after bullae, miliae and, in some cases, bullae involving the mucous membranes. In one of the families, six members had congenital dermal aplasia of the feet.