Abstract： Pemphigus is a group of life-threatening autoimmune blistering disorders. Although conventional therapies with systemic corticosteroids and immunosuppressive agents can reduce the mortality from pemphigus, complications have restricted their clinical application. With new insights into the pathogenesis of pemphigus, some novel treatment modalities, such as clearance of pathogenic antibodies in sera, biologic agents (CD20 antagonist, tumor necrosis factor-α antagonist), p38 mitogen-activated protein kinase inhibitor, cholinergic receptor agonists, CD40/CD154 blockade, desmoglein 3 synthetic peptide, etc,have been gradually introduced into the treatment of pemphigus. However, high expense and lack of reliable clinical data have limited the wide use of these new modalities in pemphigus.