Istituto Dermopatico dell'Immacolata (IDI) IRCCS, Via dei Monti de Creta 104, 00167 Rome, Italy.
Am J Clin Dermatol. 2010;11 Suppl 1:53-4. doi: 10.2165/1153427-S0-000000000-00000.
Epidermolysis bullosa is a group of inherited, chronic, non-inflammatory skin disorders, and dystrophic epidermolysis bullosa (DEB) is one of the most severe variants. The role of tumour necrosis factor alpha (TNFalpha) has not been reported in the pathogenesis of DEB. A DEB case is reported that appears to have responded well to the TNFalpha inhibitor etanercept given for the treatment of concomitant psoriatic arthritis. A progressive improvement in DEB was apparent over the first 3 months of treatment and persistent good control of DEB was noted over 3 years of therapy. A correlation between DEB improvement and etanercept has not been made, but the case may provide insight into the causal mechanisms of DEB.
大疱性表皮松解症是一组遗传性、慢性、非炎症性皮肤疾病，营养不良型大疱性表皮松解症（DEB）是最严重的类型之一。肿瘤坏死因子 α（TNFα）在 DEB 的发病机制中的作用尚未见报道。本文报告了一例 DEB 患者，似乎对 TNFα 抑制剂依那西普治疗同时发生的银屑病关节炎有良好的反应。在治疗的前 3 个月，DEB 明显逐渐改善，在 3 年的治疗过程中持续良好控制 DEB。DEB 改善与依那西普之间的相关性尚未确定，但该病例可能为 DEB 的发病机制提供了一些见解。
