| Literature DB >> 29406827 |
Christine Ahn1, Deborah Negus1, William Huang1.
Abstract
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.Entities:
Keywords: Crohn’s disease; IBD; PAPA; PAPASH; PASH; cyclosporine; inflammasome; infliximab; neutrophil; neutrophilic dermatosis; ulcerative colitis
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Year: 2018 PMID: 29406827 DOI: 10.1080/1744666X.2018.1438269
Source DB: PubMed Journal: Expert Rev Clin Immunol ISSN: 1744-666X Impact factor: 4.473