Anesthetic implications in epidermolysis bullosa dystrophica

Epidermolysis bullosa is a genetic mechanobullous disease of the stratified squamous keratinizing epithelium that affects the skin and mucous membranes. Its primary feature is the formation of blisters after minor shearing trauma to the skin or mucous membranes that can result in debilitating, even life-threatening scarring. The disease presents special problems for the anesthesia provider because the equipment used to deliver anesthesia and monitor vital signs may cause serious postoperative complications. The challenge is to maintain patency of the airway and use monitoring technology without damaging epithelial surfaces, which could result in permanent scarring. Successful anesthetic management of a patient with epidermolysis bullosa is possible if precautions with anesthetic instrumentation are observed.