天疱疮是一类罕见的自身免疫性大疱性皮肤病，具有慢性、易复发性的特征。我国天疱疮的诊治现状存在如下问题。一，早期诊断困难，常漏诊或被误诊为湿疹、脂溢性皮炎、白塞病等，患者从发病就诊至明确诊断通常延迟数月至数年；二，患者对疾病的认知不足，天疱疮患者教育存在缺口，患者接受治疗依从性不足，自行快速减药或停药引起复发的情况屡见不鲜，常导致再次治疗的敏感性下降和不良反应发生率上升；三，医师对治疗相关不良反应的管理欠缺，部分基层医院仍使用远大于疾病控制需要剂量的激素作为天疱疮的初始治疗，并存在不规范联合用药情况。2020年1月，中国医疗保健国际交流促进会皮肤科分会发布了《寻常型天疱疮诊断和治疗专家建议（2020）》，更新了我国天疱疮的诊治规范。2021年4月，中国自身免疫性大疱病协作网正式成立，旨在以多中心、多学科协作研究推动天疱疮诊疗的进一步更新。做到“早期诊断、及时治疗、精心护理、长期随访”是天疱疮诊治的原则。本文总结我国临床的天疱疮诊治现状，对比介绍国内外天疱疮诊疗的差异，并对未来发展提出思考。

Pemphigus is a rare autoimmune bullous disease with chronic and high rates of relapse. The diagnosis of pemphigus in China has following problems: first, the difficulty of early diagnosis commonly causes missed diagnosis or misdiagnosis with eczema, seborrheic dermatitis, behcet disease, ect. Patients are diagnosed clearly only after several months or years. Second, patients’ cognitive dificiency of pemphigus. Shortage of education on pemphigus patients, low acceptance and obedience and self-reduction or-withdrawal of drug causing common relapse and low sensitivity and increasing adverse reactions in retreatment. Thrid, lack of management of doctors in adverse reaction treatment. Some primary hospitals still have the overdosage of hormone to disease control and nonstandard drug combination as the primary treatment of pemphigus. In January 2020, the Dermatology Branch of China Association for the Promotion of International Exchanges in Healthcare has released Expert Recommendations on the Diagnosis and Treatment of Pemphigus Vlgaris (2020) and update the diagnosis standard of pemphigus in China. In April 2021, the China Autoimmune Bullous Disease Collaboration Network was officially established to promote the further update of pemphigus diagnosis and treatment with multi-center and multidisciplinary collaborative research. “Early diagnosis, timely treatment, careful nursing, long-term follow-up” is the principle of pemphigus diagnosis and treatment. This article concludes the situation of pemphigus in China, compares differences between home and abroad in diagnosis and treatment of pemphigus and triggers thoughts of future development.