Laryngeal manifestations of epidermolysis bullosa acquisita.
Epidermolysis bullosa acquisita is an acquired blistering skin disease caused by autoantibodies to the dermoepidermal junction of the skin. Patients present with tense bullae over areas of trauma, commonly the palms, soles, elbows, and knees. When the lesions heal, they leave a dense scar. In the head and neck, bullae in the buccal mucosa and the cervical esophagus cause strictures. To our knowledge, this is the first reported case of epidermolysis bullosa acquisita of the larynx. The patient had severe supraglottic stenosis that was caused by the trauma of a routine intubation. We describe the clinical and histologic characteristics of the disease and suggest managing the strictures with balloon dilation to minimize trauma and prevent further lesions and scarring.
Hester
J E
JE
Division of Otolaryngology-Head and Neck Surgery, Stanford University Hospital, Calif, USA.
Arnstein
D P
DP
Woodley
D
D
D000328:Adult; D003251:Constriction, Pathologic / Q000209:etiology; D016107:Epidermolysis Bullosa Acquisita / Q000150:complications* / Q000473:pathology; D006801:Humans; D007442:Intubation, Intratracheal / Q000009:adverse effects; D007818:Laryngeal Diseases / Q000209:etiology* / Q000473:pathology; D008297:Male