Reguiaï Ziad, Grange Florent
Department of Dermatology, Hôpital Robert Debré, Reims, France.
Am J Clin Dermatol. 2007;8(2):67-77. doi: 10.2165/00128071-200708020-00002.
Pyoderma gangrenosum (PG) is an ulcerative neutrophilic dermatosis seen in 1-5% of patients with inflammatory bowel disease (IBD). The pathogenesis of PG remains unclear, but may be related to abnormal T-cell responses and production of tumor necrosis factor (TNF)-alpha, a powerful proinflammatory cytokine. Although their use is not supported by appropriately controlled trials, corticosteroids and systemic immunosuppressants are the classical cornerstones of treatment of PG, against which they have a nonspecific effect. Successful curative or symptomatic treatment of associated disorders may lead to an improvement in PG. A new era for the management of chronic inflammatory disease began with the advent of biotherapies and particularly anti-TNFalpha therapy, which allows for a specific intervention in the immune cascade. Anti-TNFalpha therapy has improved and broadened the therapeutic options for IBD and, therefore, has brought new perspectives to management of the extra-intestinal manifestations of this disorder, including PG. To date, infliximab, etanercept, and adalimumab have been used in the treatment of PG. Published data have demonstrated that infliximab is highly effective in the treatment of PG, whether associated with IBD or not. This treatment is generally well tolerated, even as long-term therapy. However, rare and serious complications have been reported. Although infliximab is a costly drug, its use should be considered for patients with PG and particularly with corticosteroid-refractory PG associated with IBD. Additional comparative long-term studies are needed to determine the long-term efficacy and safety of anti-TNFalpha therapy and define its role in the management of PG, with or without accompanying IBD.
坏疽性脓皮病(PG)是一种溃疡性中性粒细胞性皮肤病,见于1%-5%的炎症性肠病(IBD)患者。PG的发病机制尚不清楚,但可能与异常的T细胞反应及肿瘤坏死因子(TNF)-α(一种强大的促炎细胞因子)的产生有关。尽管缺乏适当对照试验的支持,但皮质类固醇和全身性免疫抑制剂仍是PG治疗的经典基石,它们对PG有非特异性作用。相关疾病的成功治愈或对症治疗可能会使PG有所改善。随着生物疗法尤其是抗TNFα疗法的出现,慢性炎症性疾病的治疗进入了一个新时代,这种疗法能够对免疫级联反应进行特异性干预。抗TNFα疗法改善并拓宽了IBD的治疗选择,因此为该疾病肠外表现(包括PG)的管理带来了新的视角。迄今为止,英夫利昔单抗、依那西普和阿达木单抗已用于PG的治疗。已发表的数据表明,英夫利昔单抗无论是否与IBD相关,对PG的治疗均非常有效。这种治疗通常耐受性良好,即使作为长期治疗也是如此。然而,也有罕见且严重并发症的报道。尽管英夫利昔单抗是一种昂贵的药物,但对于PG患者尤其是与IBD相关的皮质类固醇难治性PG患者,仍应考虑使用。需要更多的长期对照研究来确定抗TNFα疗法的长期疗效和安全性,并明确其在PG管理中的作用,无论PG是否伴有IBD。