文献简介

出版社:Am J Clin Dermatol

作  者:Afsaneh Alavi •Lars E. French•Mark D. Davis•Alain Brassard•Robert S. Kirsner

编  号:

关键字:Pyoderma Gangrenosum

年  份:2017   点击量:65

文献摘要 全文翻译

Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic disorder with prototypical clinical presentations. Its pathophysiology is complex and not fully explained. Recent information regarding the genetic basis of PG and the role of auto-inflammation provides a better understanding of the disease and new therapeutic targets. PG equally affects patients of both sexes and of any age. Uncontrolled cutaneous neutrophilic inflammation is the cornerstone in a genetically predisposed individual. Multimodality management is often required to reduce inflammation, optimize wound healing, and treat underlying disease. A gold standard for the management of PG does not exist and high-level evidence is limited. Multiple factors must be taken into account when deciding on the optimum treatment for individual patients: location, number and size of lesion/ulceration(s), extracutaneous involvement, presence of associated disease, cost, and side effects of treatment, as well as patient comorbidities and preferences. Refractory and rapidly progressive cases require early initiation of systemic therapy. Newer targeted therapies represent a promising pathway for the management of PG, and the main focus of this review is the management and evidence supporting the role of new targeted therapies in PG.

坏疽性脓皮病(PG)是一种罕见的炎性嗜中性皮肤病,具有典型的临床表现。该病病理生理学复杂,尚未充分阐明。关于PG遗传基础和自身炎症作用的最新信息有助于更好理解该疾病和寻找新的治疗靶点。PG对任何年龄层的男女患者具有相同影响。皮肤中性粒细胞炎症不受控制与个体遗传易感性有关。通常需进行多模式管理以减少炎症,优化伤口愈合并治疗潜在疾病。PG管理无金标准,且高质量的循证医学证据有限。在决定个体患者的最佳治疗方式时,需考虑多种因素:皮损/溃疡的位置、数量和大小、真皮外受累、相关疾病的存在、治疗成本和治疗副作用,以及患者并发症和偏好。难治性和急性进展期患者需尽早进行系统性治疗。新的靶向治疗为PG管理提供了新途径,本综述的主要重点在管理和证明新靶向在PG治疗中的作用。