Cicatricial pemphigoid: report of five cases

Abstract

Cicatricial pemphigoid is a chronic, systemic, autoimmune disease characterized by progressive bullous skin and mucous membrane lesions that tend toward scarring and involution. Manifestations of cicatricial pemphigoid include oral mucosal bullous lesions in 85 to 90% of patients, ocular mucosal lesions in 66%, nasal mucosal lesions in 15 to 23%, and laryngeal involvement in 8 to 21%. We report five cases of cicatricial pemphigoid in which all patients had ENT manifestations--specifically, oral and nasal mucosal involvement. Three of these patients also had laryngeal lesions; one of the three had a large laryngeal ulceration and bullae that caused a laryngeal stenosis and necessitated a tracheostomy. In addition to the five case reports, we also review the literature and discuss the pathogenesis, diagnosis, and treatment of this uncommon disease.