Chinese Expert Proposal on the Diagnosis and Management of Pemphigus Vulgaris (2020)

Pemphigus vulgaris(PV)is a life-threatening autoimmune bullous disease that causes blisters and erosions on the skin and mucous membranes to standardize the diagnosis and treatment of PV,Chinese experts in this field were invited to make recommendations which are presented in this article.Pemphigus vulgaris can be divided into cutaneous mucous type,cutaneous type and mucous mucous dominant type according to the different clinical manifestations.The pathological manifestations of PV are acantholysis above the basal layer.The diagnosis of PV and the assessment of the severity of the disease are made in accordance with the clinical manifestations,histopathological features,immunofluorescence assay results,and detection of pathogenic serum antibodies.The first-line treatment of PV comprises systemic glucocorticoids.Early combination with immunosuppressive agents or rituximab is recommended for moderate and severe PV.Intravenous immunoglobulin administration is recommended for patients being treated with rituximab.Plasma exchange and stem cell transplantation can be performed if necessary.During the course of therapy,the disease activity should be closely monitored,and actions should be taken to prevent adverse reactions.