External auditory canal stricture secondary to epidermolysis bullosa

A 24-year-old woman had epidermolysis bullosa simplex involving the external ear canal with resultant stricture that led to conductive hearing loss and repeated episodes of external otitis. Treatment consisted of scar excision, bony canal enlargement, and split-thickness skin grafting. A four-year follow-up has demonstrated no recurrence of disease. Mechanobullous diseases are characterized by blistering of the skin and mucous membrane following frictional trauma. In addition, the external ear may be deformed. Intraoral scarring may result in limitation of the mouth's opening. Dysphagia may occur secondarily to esophageal scarring. Endotracheal tubation may result in postoperative blisters necessitating tracheostomy. Even surgical scrubbing and use of skin tape may lead to blister formation. The otolaryngologist should be aware of the numerous problems these patients present.