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.

Prevalence and Clinical Significance of Anti–Laminin 332 Autoantibodies Detected by a Novel Enzyme-Linked Immunosorbent Assay in Mucous Membrane Pemphigoid

“…Mucous membrane pemphigoid is associated with significant morbidity, potentially leading to definitive functional sequelae, especially in ocular or upper aerodigestive tract manifestations. 4,5 Patients with MMP exhibit, although inconsistently, circulating autoantibodies directed against various components of the dermal-epidermal/ chorioepithelial BMZ 6 including bullous pemphigoid (BP) 180 antigens [7][8][9][10] and BP230 antigens, 7,11 laminin (Lam) 332 an-tigens, [12][13][14] the 97/120-kDa linear IgA bullous disease antigen, type VII collagen, and the integrin ␤ 4 antigen. 15 The 2 major autoantigens of MMP are BP180, mainly its extracellular domain, 16,17 and Lam332; both are components of anchoring filaments and reach into the lamina densa of the BMZ.…”

Diagnosis and Management of Desquamative Gingivitis

“…Skin lesions are observed in up to 24% of MMP patients (Fleming & Korman, 2000). Fifteen percent of MMP patients experience nasal lesions, which may present in the form of crusty ulcers on the septum or turbinates (Alexandre et al, 2006;Fleming & Korman, 2000). Scarring and adhesion can also take place and may result in nasal airway obstruction (Alexandre et al, 2006;Trimarchi et al, 2009).…”

“…Scarring and an associated loss of function are the major sequelae of some forms of MMP. Life-threatening airway obstruction and sight-threatening ocular scarring have been reported (Alexandre et al, 2006;Higgins et al, 2006Higgins et al, , 2010Thorne et al, 2004;Trimarchi et al, 2009). However, scarring is rarely seen on the oral mucosa.…”

“…Fifteen percent of MMP patients experience nasal lesions, which may present in the form of crusty ulcers on the septum or turbinates (Alexandre et al, 2006;Fleming & Korman, 2000). Scarring and adhesion can also take place and may result in nasal airway obstruction (Alexandre et al, 2006;Trimarchi et al, 2009). Laryngeal MMP is a rare condition (12.2%) and the supraglottis is the most commonly affected site (Higgins et al, 2010).…”

Clinical features and diagnosis of epidermolysis bullosa acquisita

“…All squamous cell epithelia can be affected, including oral and nasal mucosa, larynx, esophagus, anal and genital mucosa as well as conjunctiva [14,[93][94][95]. While 50-65% of EBA patients have mucosal lesions [26,96,97] (Figure 5) in about 5-10% of patients, mucosal involvement predominates and patients may be classified as mucous membrane pemphigoid-variant of EBA [27,85]. In some EBA patients, exclusive involvement of the esophagus or nasal mucosa has been reported [98].…”