关键词: 天疱疮，副肿瘤性, 回顾性研究

Abstract: Zhang Yan, Pan Meng. Department of Dermatology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China Corresponding author: Pan Meng, Email: panmeng@medmail.com.cn 【Abstract】 Objective To investigate the clinical features, treatment and prognosis of paraneoplastic pemphigus （PNP）. Methods A retrospective study was performed on 11 patients with PNP hospitalized in the Department of Dermatology, Ruijin Hospital. Clinical characteristics of these patients were analyzed. Results Of the 11 patients, 10 had oral or labial erosions or ulcers, and 6 had obstructive bronchiolitis. Computed tomography （CT） showed solitary internal tumors in all the patients after appearance of skin lesions, and 8 of them were diagnosed with Castleman′s disease. All the patients had been treated with corticosteroids before operation, but achieved no obvious improvement. After 2－7 months of postoperative treatment with low-dose prednisone and thalidomide, both cutaneous and mucosal lesions healed with the relief of pulmonary symptoms in 5 patients. Conclusions Oral erosions or ulcers appear to be the most common initial manifestation of PNP with Castleman′s disease as the most frequent accompanying tumor. Early detection and timely resection of tumors are keys to successful treatment of PNP, and postoperative treatment with glucocorticoids and thalidomide proves to be effective for PNP.

Key words: Pemphigus, paraneoplasic, Retrospective studies