ESOPHAGEAL-PERFORATION IN RECESSIVE DYSTROPHIC EPIDERMOLYSIS-BULLOSA

Recessive dystrophic epidermolysis bullosa is an inherited mechanobull ous disease characterized by skin blistering and esophageal bullae. In response to minor trauma, a split in the dermis or esophageal subepit helium occurs. Esophageal perforation is a rare complication. Because the esophagus is diseased in these patients, we believe that esophagec tomy rather than repair is the treatment of choice for esophageal perf orations. We report a patient for whom early primary repair failed and esophagectomy was required.