Efficacy of combined lymphoplasma exchange for the treatment of severe refractory immune-related skin diseases: a clinical observation

Abstract

Objective To evaluate the clinical efficacy of lymphoplasma exchange (LPE) for the treatment of severe refractory immune-related skin diseases. Methods From May 2013 to October 2015, 8 patients with toxic epidermal necrolysis, drug-induced hypersensitivity syndrome (DIHS) , pemphigus vulgaris, pemphigoid or paraneoplastic pemphigus were enrolled from Department of Dermatology, Xiangya Hospital, Central South University, who showed no response to conventional therapy or presented with multiple organ dysfunction. After the treatment with LPE, the efficacy was evaluated, and adverse reactions were observed. Results After one session of LPE therapy, 6 patients received marked improvement, and were cured at last. In 1 patient with pemphigus vulgaris who was resistant to the treatment with high doses of glucocorticoids and immunosuppressive agents, the rashes regressed during the treatment with LPE, but recurred after the end of treatment. One patient with bullous pemphigoid presented with eruptive blisters on the next day after the treatment with LPE, which were considered as allergic reactions to allogeneic plasma. There were no obvious differences in white blood cell count, lymphocyte count, neutrophil count and blood platelet count in the peripheral blood of 8 patients before and after the treatment with LPE. During the follow-up of 3-5 years, all of the patients were recovered without recurrence, except 1 patient with bullous pemphigoid who died of disseminated tuberculosis after 1 year. Conclusion LPE is effective for the treatment of severe immune-related skin diseases, but attention should be paid to potential transfusion reaction and allergic reactions. Key words: Plasma exchange; Epidermal necrolysis, toxic; Pemphigus; Pemphigoid, bullous; Drug reaction with eosinophilia and systemic symptoms; Paraneoplastic pemphigus