Pemphigus is a severe autoimmune bullous dermatosis that affects the skin and/or mucosa, and it may be life-threatening without proper treatment. The guidelines and/or consensus statements for treatment vary widely between groups. We selected 6 guidelines and consensus statements established by different associations about the management of pemphigus vulgaris (PV) and/or pemphigus foliaceus (PF) to review, compare, and contrast the similarities and differences of these recommendations and provide optimal management suggestions to physicians. Corticosteroids remain a first-line therapy for pemphigus, but there are many differences in initial dose, tapering schedule, and management of relapse between different guidelines. Rituximab is a monoclonal antibody targeting CD20-positive B lymphocytes that is approved as a first-line therapy in moderate-to-severe pemphigus. Immunosuppressive agents, such as azathioprine (AZA) and mycophenolate mofetil (MMF), are also widely used as corticosteroid-sparing drugs, but the adjuvant applications and dosage regimens of different recommendations are not standardized. We attribute these differences to the clinical scoring adopted, the standards for disease severity evaluation, the publication year of each guideline, and local and regional healthcare differences.

天疱疮是一种严重的自身免疫性大疱性皮肤病，会影响皮肤和/或黏膜，如果不进行适当治疗，可能会危及生命。治疗指南和/或共识声明在各组之间差异很大。我们选择了由不同协会制定的关于寻常型天疱疮 (PV) 和/或落叶型天疱疮 (PF) 管理的 6 条指南和共识声明，对这些建议的异同进行审查、比较和对比，并为医生提供最佳管理建议。皮质类固醇仍然是天疱疮的一线治疗，但不同指南之间在初始剂量、逐渐减量计划和复发管理方面存在许多差异。利妥昔单抗是一种靶向 CD20 阳性 B 淋巴细胞的单克隆抗体，被批准作为中重度天疱疮的一线治疗药物。免疫抑制剂，如硫唑嘌呤 (AZA) 和霉酚酸酯 (MMF)，也被广泛用作皮质类固醇节约药物，但不同推荐的辅助应用和给药方案没有标准化。我们将这些差异归因于所采用的临床评分、疾病严重程度评估的标准、每个指南的发布年份以及当地和区域的医疗保健差异。