title: 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.
authors.affiliation: Stomatology Group, Division of Clinical Otolaryngology, Hospital das Clínicas, Faculty of Medicine, University of São Paulo, Brazil. miz@uol.com.br
authors.forename: Ivan Dieb
authors.initials: ID
authors.lastname: Miziara
authors.forename: Fabiana
authors.initials: F
authors.lastname: Sperandio
authors.forename: Saramira C
authors.initials: SC
authors.lastname: Bohadana
authors.forename: Natasha
authors.initials: N
authors.lastname: Braga
authors.forename: Fabrizio Ricci
authors.initials: FR
authors.lastname: Romano
authors.forename: Aroldo
authors.initials: A
authors.lastname: Miniti
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010390:Pemphigoid, Benign Mucous Membrane / Q000175:diagnosis / Q000473:pathology / Q000628:therapy