Kennedy Jeremy Scott, Devillez Richard L, Henning Jeffrey Scott
San Antonio Military Medical Center South, Lackland Air Force Base, TX 78236, USA.
J Drugs Dermatol. 2010 Jan;9(1):68-70.
Cicatricial pemphigoid (mucous membrane pemphigoid) is a group of chronic disorders characterized by recurrent subepithelial blisters and scarring, predominantly of the mucous membranes. The authors report the case of a 63-year-old female with a 20-year history of cicatricial pemphigoid who had failed treatment with cytoxan, dapsone, systemic steroids and minocycline. She achieved complete clinical remission with the addition of etanercept 50 mg twice a week with azathioprine 150 mg daily. This patient has remained clear for two years with this combination and has tolerated a tapering of the azathioprine from the initial dose of 250 mg. Etanercept is a recombinant human dimeric fusion protein which acts as a competitive inhibitor of TNF-alpha by binding to both soluble and receptor-bound molecules of TNF-alpha. There have been a few reports of successful use of TNF-alpha inhibitors in cicatricial pemphigoid. This report will discuss the possible mechanism of action of the addition of TNF-alpha inhibitors as a steroid-sparing agent in these patients.
瘢痕性类天疱疮（黏膜类天疱疮）是一组以反复出现上皮下水疱和瘢痕形成为特征的慢性疾病，主要累及黏膜。作者报告了一例63岁女性患者，患有瘢痕性类天疱疮20年，使用环磷酰胺、氨苯砜、全身性类固醇和米诺环素治疗均失败。她通过每周两次添加50mg依那西普和每日150mg硫唑嘌呤实现了完全临床缓解。该患者使用这种联合治疗方案已保持病情缓解两年，并且能够耐受硫唑嘌呤从初始剂量250mg逐渐减量。依那西普是一种重组人二聚体融合蛋白，通过与可溶性和受体结合的肿瘤坏死因子-α（TNF-α）分子结合，作为TNF-α的竞争性抑制剂发挥作用。已有一些关于在瘢痕性类天疱疮中成功使用TNF-α抑制剂的报道。本报告将讨论在这些患者中添加TNF-α抑制剂作为类固醇节约剂的可能作用机制。
