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