title: A prospective study of upper aerodigestive tract manifestations of mucous membrane pemphigoid.
abstract: We conducted a prospective study between 1995 and 2002 to investigate nose and throat (NT) manifestations of mucous membrane pemphigoid (MMP). One hundred ten consecutive patients with clinical, histologic, and immunologic criteria of MMP were seen in 2 referral centers for bullous diseases. They were systematically asked about the existence of persistent NT symptoms. Patients who had any were examined with a flexible nasopharyngolaryngoscope by the same otorhinolaryngologist. When possible, NT mucous membrane (MM) biopsies were taken for direct immunofluorescence (IF) assays to determine lesion specificity. Thirty-eight (35%) patients (23 F/15 M; mean age, 58.5 yr) had the following NT symptoms: 35 (92%) nasal, 19 (50%) pharyngeal, and 10 (26%) laryngeal. Five (13%) had acute dyspnea. Thirty-three (87%) of the 38 symptomatic patients had lesions at physical examination: 30 (79%) nasal, 6 (16%) pharyngeal, and 19 (50%) laryngeal. Laryngeal involvement was asymptomatic in 11 patients. Lesions were mainly atrophic rhinitis and oropharyngeal and epiglottal erosions. Nasal valves, choanae, pharynx, and/or larynx were severely scarred in 7 (18%) patients, causing the death of 3. Direct IF showed malpighian epithelium associated with linear immune deposits (IgG, IgA, or C3) along the chorioepithelial junction in all 18 biopsies performed, including those of 4 symptomatic patients without lesions at physical examination. The presence of severe ophthalmologic lesions (p = 0.02) and > or =3 sites involved other than NT (p = 0.02) were predictive of laryngeal involvement. In contrast, laryngeal symptoms, disease duration, HLA DQB1*0301, and smoking were not significantly associated with laryngeal lesions. In conclusion, at least 35% of MMP patients had NT involvement. Atrophic rhinitis was the most frequent lesion. The most severe were the laryngeal lesions that were significantly associated with severe ocular involvement and disseminated disease, and could be fatal. Our results highlight the necessity of a multidisciplinary approach to MMP management to assure early diagnosis of NT involvement, to guide therapeutic choices, and to improve patient survival and functional outcomes.
authors.affiliation: From Departments of Dermatology (EA 3408) (MA, FP, FC, LL, CPS) and Histology (EA 3410) (MH, NL, CPS), Avicenne Hospital-UFR Paris 13, Bobigny; Departments of Dermatology (MA, PT, LD, CPS), Otorhinolaryngology (MDB), and Stomatology (FP), Saint-Louis Hospital, Paris; Department of Pathology (SF), Necker Enfants-Malades Hospital, Paris; and Department of Ophthalmology (SD), Bichat Hospital, Paris, France.
authors.forename: Marina
authors.initials: M
authors.lastname: Alexandre
authors.forename: Marie-Dominique
authors.initials: MD
authors.lastname: Brette
authors.forename: Francis
authors.initials: F
authors.lastname: Pascal
authors.forename: Paul
authors.initials: P
authors.lastname: Tsianakas
authors.forename: Sylvie
authors.initials: S
authors.lastname: Fraitag
authors.forename: Serge
authors.initials: S
authors.lastname: Doan
authors.forename: Frédéric
authors.initials: F
authors.lastname: Caux
authors.forename: Alain
authors.initials: A
authors.lastname: Dupuy
authors.forename: Michel
authors.initials: M
authors.lastname: Heller
authors.forename: Nicole
authors.initials: N
authors.lastname: Lièvre
authors.forename: Virginia
authors.initials: V
authors.lastname: Lepage
authors.forename: Louis
authors.initials: L
authors.lastname: Dubertret
authors.forename: Liliane
authors.initials: L
authors.lastname: Laroche
authors.forename: Catherine
authors.initials: C
authors.lastname: Prost-Squarcioni
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016009:Chi-Square Distribution; D004724:Endoscopy; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D007818:Laryngeal Diseases / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009668:Nose Diseases / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D010390:Pemphigoid, Benign Mucous Membrane / Q000150:complications* / Q000276:immunology / Q000473:pathology; D010608:Pharyngeal Diseases / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D011446:Prospective Studies; D014057:Tomography, X-Ray Computed