Recent advances in managing and understanding pyoderma gangrenosum.

Josh Fletcher,Afsaneh Alavi,Raed Alhusayen

doi:10.12688/f1000research.19909.1

Abstract

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.

Highlights

Pyoderma gangrenosum (PG) is a rare auto-inflammatory ulcerative dermatosis with an overall incidence of 5.8 per 100,000 individuals and an increased mortality rate when compared with the general population[1,2]
Wound care, and compression therapy are all important tenets in the management of PG
It is important to note that owing to the rarity of this condition, there are very few randomized controlled trials (RCTs)

Summary

Introduction

Pyoderma gangrenosum (PG) is a rare auto-inflammatory ulcerative dermatosis with an overall incidence of 5.8 per 100,000 individuals and an increased mortality rate when compared with the general population[1,2]. One patient in this study had new-onset rapidly progressive genital ulcers, likely representing PG at a different location[19] Gevokizumab, another monoclonal antibody targeting IL-1β, showed promise in the treatment of PG; the rights to this drug were sold in 20168. Case reports in the literature demonstrate either partial or complete resolution of PG lesions with ustekinumab; more studies are needed to confirm efficacy[6,8]. Three patients with treatment-resistant PG and both comorbid Crohn’s disease and inflammatory arthritis were given tofacitinib, leading to complete resolution in two patients and symptom improvement in the third by 12 weeks[45]. Mental health support is warranted given the association between PG and major depressive disorder[27]

Conclusions

Cohen PR

Findings

23. Mejia LM