St George Hospital, Gray Street, Kogarah, Sydney, NSW 2217, Australia.
Dermatol Clin. 2011 Oct;29(4):607-12. doi: 10.1016/j.det.2011.06.016. Epub 2011 Aug 15.
Paraneoplastic pemphigus (PNP) or paraneoplastic autoimmune multiorgan syndrome (PAMS) is a life-threatening autoimmune blistering disease commonly associated with lymphoproliferative neoplasms. This article focuses on current management strategies in PNP/PAMS, and reported instances of their treatment successes and failures. Due to the rarity of the condition and the high rates of treatment failure, no randomized control trials exist to guide the evidence-based treatment of this condition; all evidence to date on the efficacy of therapeutic modalities has been gained from individual case reports, small case series, and expert recommendations.
副肿瘤天疱疮（PNP）或副肿瘤自身免疫多器官综合征（PAMS）是一种危及生命的自身免疫性水疱病，通常与淋巴增生性肿瘤有关。本文重点介绍了 PNP/PAMS 的当前治疗策略，以及报告的治疗成功和失败案例。由于这种疾病的罕见性和高治疗失败率，目前尚无随机对照试验来指导这种疾病的循证治疗；迄今为止，关于治疗方法疗效的所有证据都是通过个案报告、小病例系列和专家建议获得的。
