PYODERMA GANGRENOSUM WORKGROUP
Pyoderma gangrenosum (PG) is a painful ulcerative skin disease with an incidence of 0.3 to 1.0/100.000. The lack of consensus and concordance of existent diagnostic criteria seems to not have addressed the current misdiagnosis rate of PG, making problematic the estimation of an accurate prevalence. PG is an inflammatory neutrophilic dermatosis with five described clinical subtypes and poorly understood pathogenesis. Strong associations with systemic illness (50-74%) have been identified; of these, inflammatory bowel disease (IBD, 20-65%), inflammatory arthropathies (12-20%), hematologic dyscrasia (4-12%), solid organ neoplasia (2-4%) and hidradenitis suppurativa (1-6%) have been established consistently in retrospective studies and systematic reviews.
Treatment options range from topical to systemic corticosteroids, cyclosporine, and more recently biologics with variable efficacy depending on clinical context. Due to the scarcity of validated outcome measurement instruments for PG as well as the limited number of clinical trials and randomized controlled trials (RCTs) the treatment options are very limited. Studies relating to PG are also challenged by the lack of a PG-specific severity scale and validated tools for assessing the effectiveness, efficacy, and safety of therapeutics. There is a need for a uniform therapeutic standard of care. As of today there is no consensus on the core outcome domains and core outcome measurement instruments for PG and there is a lack of content validity of instruments for PG due to the absence of validation studies.
PG working group would consist of a group of experts with two different goals: 1) to develop proper outcome measures to assess the impact of PG therapy as well as to test its validity in clinical grounds 2)to identify a determine a core domain set to be collected when assessing patients with PG. We hope to form a working group of experts as listed above who all have clinical and research expertise in the diagnosis and management of this skin condition.
1.Lu JD, Hobbs MM, Huang WW, Ortega-Loayza AG, Alavi A.Identification and evaluation of outcome measurement instruments in pyodermagangrenosum: a systematic review.Br J Dermatol. 2020 Mar 11. doi: 10.1111/bjd.19027. [Fletcher J, Alhusayen R, Alavi A.Recent advances in managing and understanding pyoderma gangrenosum.F1000Res. 2019 Dec 12;8. pii: F1000 Faculty Rev-2092. doi: 10.12688/f1000research.19909.1. eCollection 2019. Review.