关键词: 血浆置换； 表皮坏死松解症, 中毒性； 天疱疮； 类天疱疮, 大疱性； 药物超敏反应综合征； 副肿瘤性天疱疮

Abstract: 【Abstract】Objective To discuss the clinical efficacy and adverse reactions of lymphoplasma exchange of refractory severe immune related dermatosis such as Toxic epidermal necrolysis、DRESS syndrome、pemphigus vulgaris、bullous pemphigoid、paraneoplastic pemphigus and to analyze the potential mechanism. Methods Clinical and laboratory data before and after lymphoplasma exchange(LPE) were collected from 8 patients, who were conventional therapy invalid or with multiple organ function impaired, with different severe immune related dermatosis in the Department of Dermatology of Xiangya Hospital from May 2013 to October 2015, analyzed retrospectively, to evaluate efficacy of LPE and to observe the adverse reaction. Results Symptoms had been significantly improved in six of eight patients and eventually recovered; one patient was resisted to high-dose glucocorticoids and immunosuppressive therapy and the rash was relieved during LPE but was repeated after treatment; the patient with bullous pemphigoid showed the explosive blister on the next day after LPE treatment, and we considered it as an allogeneic plasma allergic reaction. Conclusion Lymphoplasma exchange(LPE) is effective for the treatment of severe autoimmune related dermatosis, especially for those patients who were resisted to high-dose glucocorticoids and immunosuppressive therapy. LPE can be chosen as the preferred treatment for patients with multi-organ damaged. Furthermore, potential transfusion reaction and allergic reactions should be noticed.

Key words: Plasma exchange, Epidermal necrolysis, toxic, Pemphigus, Pemphigoid, bullous, Drug reaction with eosinophilia and systemic symptoms, Paraneoplastic pemphigus