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关键字:坏疽性脓皮病

年  份:2019   点击量:31

文献摘要 全文翻译

Pyoderma Gangrenosum (PG) is a rare neutrophilic dermatosis with multiple different clinical presentations and associated comorbidities. PG has historically been a challenging disorder to diagnose, leading to the development of new diagnostic criteria rather than the traditional approach of a diagnosis of exclusion. The pathophysiology is thought to involve both innate and adaptive immune system dysregulation, neutrophilic abnormalities, environmental, and genetic factors. As of today, no gold standard therapy exists for the treatment of PG, and the literature is restricted to mainly case reports, case series, and 2 small randomized clinical trials. Topical, systemic, and biologic therapy, as well as adequate analgesia and proper wound care all play a role in the management of PG. Recent studies have identified additional cytokines and signalling cascades thought to be involved in the pathogenesis of PG, ultimately leading to the development of new targeted therapies. This review will focus on recent advances in the pathophysiology, clinical presentation and associated comorbidities, diagnosis, and management of PG.

坏疽性脓皮病(PG)是一种罕见的嗜中性皮肤病,具有多种不同的临床表现和相关并发症。从历史上看,PG一直是一种难以诊断的疾病,从而导致新的诊断标准的发展,而非传统排除诊断的发展。据认为,病理生理学受先天和适应性免疫系统失调、嗜中性粒细胞异常、环境和遗传因素影响。截止目前,治疗PG尚无黄金疗法,且文献仅限于主要病例报告、病例系列和2项小型随机临床试验。局部、全身和生物治疗,以及充分无痛和适当的伤口护理,均在PG管理中发挥作用。最近研究发现,其他细胞因子和信号级联影响PG发病机制,最终导致新的靶向疗法的发展。最近的研究已经确定了与PG发病机制有关的其他细胞因子和信号传导级联反应,最终导致了新的靶向疗法的发展。本综述侧重介绍PG病理生理学、临床表现、相关并发症以及诊断和管理方面的最新进展。