Pyoderma gangrenosum: a review of pathogenesis and treatment

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.

KEYWORDS:

• Crohn’s disease
• cyclosporine
• IBD
• inflammasome
• infliximab
• neutrophilic dermatosis
• neutrophil
• PAPA
• PAPASH
• PASH
• ulcerative colitis

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties. Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This article was not funded.