Head and neck manifestations of epidermolysis bullosa

Although considerable attention has focused on the pathophysiology and external dermatologic consequences of epidermolysis bullosa (EB), head and neck mucosal manifestations pose specific management problems. The otolaryngologic aspects of EB, with emphasis on involvement and treatment of the ears, oral cavity and teeth, larynx and trachea, and pharynx and esophagus, are reviewed. Guidelines are presented for specialized dental, anesthetic, and nutritional management in patients with this potentially fatal disease.