Management of mucous membrane pemphigoid
- Kim B Yancey, MD
Kim B Yancey, MD
- Professor & Chair, Department of Dermatology
- University of Texas Southwestern Medical Center in Dallas
Mucous membrane pemphigoid (MMP) is a rare, chronic, autoimmune, subepithelial blistering and erosive disease that affects the mucosal surfaces of the mouth (gingiva, movable mucosa, tongue, and palate), eyes, nose, nasopharynx, hypopharynx, larynx, esophagus, genitals, and/or anus (picture 1A-C) [1,2]. Limited cutaneous involvement (typically localized to the head, neck, or upper trunk) also may be present. (See "Clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid", section on 'Mucous membrane pemphigoid'.)
MMP may cause significant morbidity due to the tissue destruction and functional limitations that result from chronic mucosal inflammation, pain, and scarring. As examples, gingival inflammation and scarring may lead to gingival recession and loss of teeth, conjunctival scarring may contribute to blindness, and laryngeal scarring may result in airway loss.
The treatment of MMP, particularly MMP involving the oral cavity, will be reviewed here. The management of ocular MMP (ocular cicatricial pemphigoid) and the clinical manifestations and diagnosis of MMP are discussed separately. (See "Ocular cicatricial pemphigoid" and "Epidemiology and pathogenesis of bullous pemphigoid and mucous membrane pemphigoid" and "Clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid" and "Management and prognosis of bullous pemphigoid".)
APPROACH TO TREATMENT
The primary goals of the treatment of mucous membrane pemphigoid (MMP) are to halt the progression of the disease, improve symptoms, and prevent adverse sequelae of chronic tissue inflammation and scarring [3,4]. Few high quality trials have evaluated interventions for MMP . Thus, treatment is largely guided by clinical experience and small uncontrolled studies. Consistent use of the recommended measures for disease activity and treatment outcomes published in 2014 may aid in the systematic interpretation of data from future studies .
The approach to therapy is influenced by the following clinical factors :
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- Le Roux-Villet C, Prost-Squarcioni C, Alexandre M, et al. Rituximab for patients with refractory mucous membrane pemphigoid. Arch Dermatol 2011; 147:843.
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- APPROACH TO TREATMENT
- ORAL DISEASE
- Mild disease
- - Topical corticosteroids
- Side effects
- - Topical tacrolimus
- - Intralesional corticosteroids
- Moderate to severe disease
- - Systemic glucocorticoids
- - Dapsone
- Severe and refractory disease
- - Immunosuppressants
- - Biologic therapies
- Intravenous immune globulin
- Other therapies
- Adjunctive measures
- EXTRAORAL DISEASE
- SUMMARY AND RECOMMENDATIONS