Dramatic improvement of pyoderma gangrenosum with infliximab in a patient with PAPA syndrome
Dramatic improvement of pyoderma gangrenosum with infliximab in a patient with PAPA syndrome
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DOI：
10.1111/j.1525-1470.2005.22320.x
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发表时间：
2005-05-01
影响因子：
1.5
通讯作者：
Pascual, V
中科院分区：
文献类型：
--
作者：
Stichweh, DS;Punaro, M;Pascual, V
英夫利西单抗是一种嵌合抗肿瘤坏死因子α单克隆抗体（抗TNF α），最近已被证明对炎症性肠病相关的坏疽性脓毒症具有有益作用。患有化脓性无菌性关节炎、坏疽性化脓性坏疽和痤疮三联征（一种由CD 2结合蛋白-1（CD 2BP 1）基因突变引起的自身炎症过程）的患者可能患有严重的坏疽性化脓性坏疽。我们描述了一名患有这种综合症的14岁患者，他对多种治疗均无反应。在一次英夫利西单抗输注后，观察到坏疽性脓疡的显著改善，第二次输注导致其消退。我们的观察将英夫利西单抗的治疗用途扩展到PAPA综合征的这一组成部分。
Infliximab, a chimeric antitumor necrosis factor alpha monoclonal antibody (anti-TNF alpha), has been recently shown to have a beneficial effect on pyoderma gangrenosum associated with inflammatory bowel disease. Patients with the syndromic triad of pyogenic sterile arthritis, pyoderma gangrenosum, and acne, an autoinflammatory process caused by mutations in the CD2 binding protein-1 (CD2BP1) gene, can have severe pyoderma gangrenosum. We describe a 14-year-old patient with this syndrome who was unresponsive to multiple therapies. A dramatic improvement in his pyoderma gangrenosum was observed after one infusion of infliximab, and a second infusion led to its resolution. Our observation extends the therapeutic use of infliximab to this component of PAPA syndrome.