Mucous membrane pemphigoid; Etiology, diagnosis and treatment

Abstract

AbstractMucous membrane pemphigoid (MMP) is a chronic autoimmune subepithelial vesiculobullousdisorder that predominantly affects the mucous membranes more frequently than the skin. Severaltarget antigens in basement membrane zone have been identified in MMP. It is characterized bylinear deposition of IgG, IgA or C3 along the basement membrane zone. The disease severity andextension is highly variable. The patients may present with only mucosal or skin lesions or combinedmultiple sites. In the oral cavity, the most frequently affected site is the gingiva presented asdesquamative gingivitis. The diagnosis of MMP is mainly based on clinical findings, histopathologicand immunofluorescence features. There is no gold standard therapy for MMP. The treatmentshould be individualized based on the sites of involvement, clinical severity and disease progression.Corticosteroids and immunosuppressive agents are the mainstay of treatment. The significantcomplication is scarring of the oropharyngeal and ocular mucous membranes which can lead tostrictures and blindness. Multidisciplinary approach is necessary for the diagnosis and managementof MMP. This article reviews the epidemiology, pathophysiology, clinical presentation, diagnosis andtreatment of MMP.Keywords: Autoimmune, Mucous membrane pemphigoid, Corticosteroids, Diagnosis, Treatment