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Mucous membrane pemphigoid uncommon disease in oral cavity and synonymous with cicatricial pemphigoid. This disease is caused by autoimmune reaction that autoantibody reacts antigen located in basement membrane and epithelium is separated from underlying connective tissue. It affects female over sixth decade, commonly. Oral mucosa, especially gingiva is common site but conjunctival, nasal, pharyngeal, laryngeal, esophageal, varginal mucosa and skin are involved. Intraoral findings show Nikolsky sign, irregular erythema, erosion, vesicle, and ulceration at mucous membrane. To differentiate from diseases of positive Nikolsky sign, should perform histologic, immunologic test. Histologic features show subbasilar cleft and direct immunologic features show IgG, C3 deposits at basement membrane in linear pattern. Mucous membrane pemphigoid is incurable disease because symptoms are repetitively improved or worsed for several years. Patients are commonly managed with topical and systemic steroid. To avoid side effects of prolonged steroid therapy and to maintain immunosupressive effects, combination therapy of azathioprine with steroid is effective. This case reports that mucous membrane pemphigiod is diagnosed based on clinical and histologic teatures, is treated with topical, systemic steroid and azathioprine therapy.
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