Junctional Epidermolysis Bullosa of the Larynx

Abstract

Epidermolysis bullosa (EB) is a group of rare inherited disorders in which minor trauma causes blister formation in the skin and mucosa, including the esophagus. Morbidity varies with the type of disease and ranges from occasional trivial skin blisters to death in infancy. Laryngeal involvement presenting as hoarseness and respiratory distress has been reported in nine patients, five of whom had junctional EB. We present the sixth case of junctional EB with laryngeal involvement, and offer guidelines for otolaryngologists and anesthesiologists caring for these fragile patients.

Twelve hour anaesthesia in a patient with epidermolysis bullosa

“…Possible airway obstruction and laryngeal stenosis can occur from these bullae. 9 The patients pose a particular challenge to the anaesthetist, both in airway management as well as in maintenance of skin integrity. Several anaesthetic case reports have been documented.…”