Abstract: Mucosal pemphigoid is a rare subepidermal immune bullous disease, mainly involving the oral cavity, eyes and other mucous membranes, with the risk of scarring, which can be life-threatening in severe cases. For patients with mucosal pemphigoid, early diagnosis and early treatment can avoid serious complications. For mild patients, dapsone or topical glucocorticoids can be used; for severe patients, combination of immunosup-pressants helps to improve clinical efficacy and reduce the dosage of glucocorticoid, including cyclophosphamide, azathioprine, methotrexate, and mycophenolic acid esters. For some refractory cases without any response to the treatments of dapsone, glucocorticoids and immunosuppressants, biological agents (including rituximab, etanercept, infliximab, and braziltinib), intravenous immune globulin and other therapies can be selected. For some patients with mucosal scarring, surgery or laser therapy can be considered to improve symptoms. However, it is necessary to further demonstrate the clinical efficacy of some immunosuppressants and biologic agents on mucosal pemphigoid in a lot of larger clinical trials. 

Key words: mucosal pemphigoid, drug therapy, surgery