关键词: 天疱疮, 诊断, 治疗, 专家建议

Abstract: 【Abstract】 Pemphigus vulgaris is a kind of autoimmune bullous disease which seriously threatens human health. In order to standardize the diagnosis and treatment of this disease, domestic famous experts in this field were invited to make the recommendations after rigorous discussion. According to clinical manifestations, pemphigus vulgaris can be divided into cutaneous-mucosal type, cutaneous type and mucosal-dominant type. Its pathological manifestations are characterized by acantholysis above the basal layer. According to clinical manifestations, histopathological features, immunofluorescence findings and serum anti-desmoglein antibody levels, the diagnosis of pemphigus vulgaris can be made. Systemic glucocorticoids are the first-line treatment, and the doses of glucocorticoids should vary with disease severity. Early combination with immunosuppressive agents or rituximab is recommended for the treatment of patients with moderate or severe pemphigus vulgaris, and intravenous immunoglobulin is recommended to be combined with biological agents. If necessary, plasma exchange and stem cell transplantation can be performed. In the course of treatment, changes of patients′ condition should be closely observed to reduce the occurrence of adverse reactions.

Key words: Pemphigus, Diagnosis, Therapy, Expert proposal