Abstract
Pyoderma gangrenosum (PG) is a rare, and often challenging to diagnose, inflammatory disorder with relatively high rates of morbidity and mortality. Central to the diagnosis of PG is histologic evaluation and exclusion of other entities. Large-scale studies investigating the proportion of patients receiving a thorough diagnostic work-up, as well as prevalence studies regarding comorbidities and systemic treatment in PG using claims-based data, are sparse. Our objective was to identify patients diagnosed with PG and describe the diagnostic work-up and prevalence of common comorbidities and therapies in this population using claims-based data in a retrospective cohort study. In order to better understand practices of diagnostic work-up, we captured rates of skin biopsy, tissue culture, and/or surgical debridement prior to initial diagnosis. We also identified the prevalence of PG-associated comorbidities and initial immunosuppressive therapy given for PG. Of the 565 patients diagnosed with PG, 9.4% underwent skin biopsy, 8% tissue culture, and 1.4% both skin biopsy AND tissue culture prior to diagnosis. Inflammatory bowel disease was the most prevalent comorbidity (16.3%). The most common treatment administered was systemic corticosteroids (17%). Although practice guidelines explicitly delineate histology and exclusion of infection as important diagnostic criteria, only a minority of patients in this study underwent skin biopsy and/or tissue culture prior to receiving a diagnosis of PG, suggesting that patients may receive a diagnosis of PG without having tissue evaluation. Such discordance between practice guidelines and “real-world” practice inevitably increases the risk for misdiagnosis of PG and misdirected treatment with immunosuppressants for presumptive PG in cases of PG mimickers. Moreover, comorbidities associated with PG may occur, or be identified in, a lower proportion of patients as compared with what is reported in the existing literature. Study limitations include a population restricted to < 65 years with commercial insurance and the reliance upon ICD diagnostic coding to capture the population.
Data availability
Supplementary tables provided.
Code availability
Not applicable.
References
Su WP, Davis MD, Weenig RH, Powell FC, Perry HO (2004) Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria. Int J Dermatol 43(11):790–800
Jockenhöfer F, Wollina U, Salva KA et al (2019) The PARACELSUS score: a novel diagnostic tool for pyoderma gangrenosum. Br J Dermatol 180(3):615–620
Maverakis E, Ma C, Shinkai K et al (2018) Diagnostic criteria of ulcerative pyoderma gangrenosum: a Delphi consensus of international experts. JAMA Dermatol 154(4):461–466
Binus AM, Qureshi AA, Li VW, Winterfield LS (2011) Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients. Br J Dermatol 165(6):1244–1250
Kakkenberger BH, Hinton A, Krishna SG (2018) The impact of underlying disease state on outcomes in patients with pyoderma gangrenosum: a national survey. J Am Acad Dermatol 79(4):659–663
Sasor SE, Soleimani T, Chu MW et al (2018) Pyoderma gangrenosum demographics, treatments, and outcomes: an analysis of 2273 cases. J Wound Care 27(Sup1):S4–S8
Xu A, Balgobind A, Strunk A, Garg A, Alloo A (2019) Prevalence estimates for pyoderma gangrenosum in the United States: an age- and sex-adjusted population analysis. J Am Acad Dermatol. https://doi.org/10.1016/j.jaad.2019.08.001
Lockwood SJ, Di DG, Butler D et al (2018) The validity of the diagnostic code for pyoderma gangrenosum in an electronic database. Br J Dermatol 179(1):216–217
Weenig RH, Davis MDP, Dahl PR, Su WPD (2002) Skin ulcers misdiagnosed as pyoderma gangrenosum. N Engl J Med 347(18):1412–1418
Ashchyan HJ, Butler DC, Nelson CA et al (2018) The association of age with clinical presentation and comorbidities of pyoderma gangrenosum. JAMA Dermatol 154(4):409–413
Borda LJ, Jaller JA, Kirsner RS (2019) Absence of donor-site pathergy following fractional autologous full-thickness skin grafting in pyoderma gangrenosum. Br J Dermatol 181(4):847–848
Haag CK, Bacik L, Latour E, Morse DC, Fett NM, Ortega-Loayza AG (2020) Perioperative management of pyoderma gangrenosum. J Am Acad Dermatol. https://doi.org/10.1016/j.jaad.2020.01.002
Langan SM, Groves RW, Card TR et al (2012) Incidence, mortality, and disease associations of pyoderma gangrenosum in the United Kingdom: a retrospective cohort study. J Invest Dermatol 132:2166–2170
Kaffenberger BH, Hinton A, Krishna SG (2018) The impact of underlying disease state on outcomes in patients with pyoderma gangrenosum: a national survey. J Am Acad Dermatol 79(4):659–663
Funding
This project was accomplished through a generous gift from the Louis and Rachel Rudin Foundation, Inc. that supports the research education of residents at NYU Langone Health’s Ronald O. Perelman Department of Dermatology.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors have no conflicts of interest to report.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Supplementary Information
Below is the link to the electronic supplementary material.
Rights and permissions
About this article
Cite this article
Shaigany, S., Wong, P.W., Caplan, A. et al. Diagnostic work-up and treatment in patients with pyoderma gangrenosum: retrospective analysis of US insurance claims-based data. Arch Dermatol Res 315, 95–99 (2023). https://doi.org/10.1007/s00403-021-02278-z
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00403-021-02278-z