[Recessive dystrophic epidermolysis bullosa (with esophageal involvement)]

We are presenting a young man with Epidermolysis Bullosa (Dystrophica recessive type) who had lesions in hands, feet and face; some plaques of alopecia were noted on the scalp. Three years ago the patient showed marked dysphagia due to some esophagus erosions, which have been appearing regularly. One year ago it was necessary to take the patient to the Hospital where a esophagectomy was performed replacing the esophagus by a piece of colon.