Abstract: 【Abstract】 Pemphigus is an autoimmune bullous disease, and paraneoplastic pemphigus （PNP） is a unique subtype of pemphigus. Clinical characteristics of PNP mainly include severe and refractory painful erosions and ulcers of the mucosa, polymorphous skin lesions, and comorbid neoplasms, often accompanied by other systemic involvement （such as bronchiolitis obliterans）. Most patients respond poorly to treatment, and in severe cases, it is life-threatening. The diagnosis and treatment of PNP involves dermatology, stomatology, oncology, hematology, respiratory medicine, surgery, anesthesiology, general internal medicine, intensive care unit and other departments. Relevant academic organizations enrolled experts in related fields together to formulate this consensus, to provide guidance for the diagnosis and treatment of PNP. Chinese standards for PNP diagnosis are proposed based on the status of diagnosis and treatment of PNP in China. Treatment suggestions are put forward from the aspects of skin and mucosal involvement, neoplasms, bronchiolitis obliterans, and complications.

Key words: Pemphigus, Paraneoplastic pemphigus, Neoplasms, Bronchiolitis obliterans, Rare disease, Diagnosis, Treatment, Expert consensus