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REN Fa-liang, HUANG Dan, ZHENG Yun-peng, GU Heng*. *Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China Corresponding author: GU Heng��Email: guheng@aliyun.com ��Abstract�� Immunosuppressive agents are often combined to treat patients with moderate to severe pemphigus vulgaris resistant to systemic glucocorticoids. Cyclophosphamide in combination with dexamethasone pulse therapy has been widely used and proven effective in clinical trials. It has been indicated that cyclophosphamide is superior to cyclosporine and azathioprine with a steroid-sparing benefit. Oral cyclophosphamide is not recommended. At present, there is a lack of reasonably designed randomized controlled clinical trials on this subject in China. Long-term follow-up is required to evaluate its side effects, such as complicated infections, carcinogenicity, and impaired fertility. Based on relevant literature, especially reports on randomized controlled clinical trials, guidelines on diagnosis and treatment, and systematic reviews, the authors review the usage of, clinical evaluation of, as well as manifestations and prevention of adverse reactions to, cyclophosphamide alone or in combination with dexamethasone pulse therapy. ��Key words�� Pemphigus vulgaris; Cyclophosphamide