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Mucous membrane pemphigoid is a heterogeneous subepithelial blistering disease predominantly affecting oral, ocular mucous membrane and occasionally the skin.
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Like other forms of pemphigoid, the disorder is characterized by the formation of autoantibodies against structural proteins of the dermal-epidermal junction.
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Early diagnosis is critical and immunosuppressive treatment may prevent scarring.
Mucous Membrane Pemphigoid
Section snippets
Key points
Epidemiology
The true incidence of MMP is unclear. A recent study from the United Kingdom demonstrated that ocular MMP accounted for 61% of the cases of newly diagnosed cicatricial conjunctivitis and the incidence was calculated as 0.8 per million population.16 The incidence of MMP was estimated to be 1.3 to 2.0 per million per year in France and Germany.17, 18 MMP predominantly affects women more often than men with a female-to-male ratio of nearly 2:1.19 MMP mainly occurs in the elderly population,
Pathogenesis
The pathogenesis of MMP is complex. MMP has been found to be heterogeneous with several different antigens implicated. The pathogenic relevance of autoantibodies in MMP has been demonstrated in vivo and in vitro.
Circulating IgG and/or IgA autoantibodies against components of the BMZ found in the serum of patients with MMP indicate that MMP is mediated by a humoral immune response.24, 25 Loss of immunologic tolerance to structural proteins in the BMZ results in development of autoantibodies. By
Clinical presentation
MMP can affect multiple mucosal sites, occasionally with skin involvement. It is a chronic, progressive condition that most frequently involves the oral mucosa (85% of patients), followed by ocular conjunctiva (65%), nasal mucosa (20%–40%), skin (25%–30%), anogenital area and/or pharynx (20%), larynx (5%–15%), and esophagus (5%–15%) (Fig. 2, Fig. 3, Fig. 4, Table 2).37, 38 Lesions occurring at any site may heal with scarring.
There is a great variability in the presentation and severity among
Diagnostic Criteria
The first international consensus statement on MMP recommended the diagnostic criteria must be based on clinical presentation as well as the presence of certain immunopathologic features (Table 3).1
Oral lesions usually involve the palate and gingival areas, and also the labial, tongue, and buccal mucosa. The lesions manifest as erythema, erosions, pseudomembrane, and sometimes intact blisters. Frequently the gingival lesions are descriptively referred to as desquamative gingivitis, which can
Management Goals
The outcome of MMP therapy is related to the involved sites and early treatment. Involvement of ocular esophageal, genital, and laryngopharyngeal mucosa is typically associated with irreversible scarring. The scarring process may be prevented or retarded by early appropriate interventions. The primary goal in the treatment of MMP is to prevent the progression to blindness, strictures, and airway obstruction, thus preserving function and preventing disability.
Large randomized controlled clinical
Summary
MMP is chronic and frequently associated with exacerbations and remissions of clinical signs and symptoms. Clinicians should use pathologic and immunonologic techniques to help diagnose patients. Multidisciplinary collaboration is often necessary for the diagnosis and proper treatment of MMP. Systemic adjuvant immunosuppressive therapy is necessary for patients with progressive disease. In spite of the advances in available immunosuppressive medications and biologics, scarring is a significant
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2022, Autoimmunity ReviewsCitation Excerpt :Oral mucosa is usually the initial site of MMP and gingival lesions are with 80% the most common oral manifestation [30,132]. Patients often complain of soreness, pain, in particular with food intake, bleeding, and peeling sensation [33,133]. Desquamative gingivitis is the main oral feature of MMP, but MMP is not the only cause of desquamative gingivitis[134–136] (Fig. 3).
Conflict of Interest: The authors have nothing to disclose.
This material is supported by the National Institutes of Health, including NIH K24-AR 18 02207 (Werth), by the Department of Veterans Affairs, Veteran Health Administration, Office of Research and Development, Biomedical Laboratory Research and Development and by Innovative Research Teams in Universities, Liaoning Bureau of Education (LT2012012).
A version of this article appeared as Sollecito TP, Parisi E. Mucous membrane pemphigoid. Dent Clin N Am 2005;49:91–106.