Pyoderma gangrenosum: a review of pathogenesis and treatment

坏疽性脓皮病 医学 发病机制 皮肤病科 病理 疾病
作者
Christine Ahn,Deborah Negus,William W. Huang
出处
期刊:Expert Review of Clinical Immunology [Taylor & Francis]
卷期号:14 (3): 225-233 被引量:131
标识
DOI:10.1080/1744666x.2018.1438269
摘要

Introduction: Pyoderma gangrenosum (PG) is a complex neutrophilic dermatosis that can occur as an idiopathic disease, in association with systemic conditions such as inflammatory bowel disease, as part of an inherited inflammatory syndrome. It can be challenging to treat, as it occurs in a wide variety of clinical settings and there is a lack of a standardized treatment approach. The main limitations to treatment have been an incomplete understanding of the pathogenesis. However, recent advances have been made in understanding the pathogenesis of this condition, and PG is now considered an autoinflammatory disease process.Areas covered: This review discusses the newest studies that further define our understanding of this disease and the relevant literature on treatment options for pyoderma gangrenosum.Expert commentary: The presence of abnormal neutrophils and T-cells lead to immune dysregulation, leading to lesions of PG. Increased levels of inflammatory mediators including IL-1β, IL-8, IL-17, and TNF-α contribute to the development of the disease but there are still several unknown factors, including the trigger for immune dysregulation and additional contributory components of the immune system. We provide our approach to the management of PG lesions, which involves a multi-faceted approach including wound care, topical therapy, and systemic medications in most cases.
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