Bullous pemphigoid with the unusual complication of tracheobronchial involvement.

Abstract

Bullous pemphygoid is the most common blistering skin disease, characterized by an autoantibody response against two major hemidesmosomal antigens within the dermo-epidermal junction. We describe a proven case of bullous pemphigoid with extensive tracheobronchial involvement and with the only bronchoscopic images available in the published literature, to our knowledge. The patient, a 73-year-old woman with a medical history of bullous pemphigoid, was admitted to our hospital for dyspnea, productive cough, and blood-streaked sputum. She underwent bronchoscopy, which showed ulcerative tracheitis with fibrinous exudates. After antibiotic therapy, a repeat bronchoscopy revealed hemorrhagic vesciculobullous lesions in the subglottic area and at the level of the main bronchi. Pathologic evaluation, direct immunofluorescence microscopy examination, and enzyme-linked immunosorbent assay led to a definitive diagnosis of bullous pemphigoid. Due to the potential confounding presence of bacterial superinfection, the real prevalence of such manifestation of this disease is still unknown. Our experience should alert clinicians about this possible localization of bullous pemphigoid.