Management of airway obstruction in cicatricial pemphigoid

Cicatricial pemphigoid is a chronic vesiculobullous disease of the mucosal epithelium that primarily involves the oral cavity and the eyes. The clinical and histologic features are identical to those of bullous pemphigoid, and these features often can be nonspecific for other disease processes. It is not unusual for a period of 1 year or more to elapse before a diagnosis is made. The diagnosis of cicatricial pemphigoid requires characteristic lesions and histopathologic evidence of immunoglobulin deposition along the basement membrane, as well as a high index of suspicion. The authors detail a case of cicatricial pemphigoid resulting in airway obstruction and present the treatment required for both stabilization of the airway and resolution of the disease process.