The validity of the diagnostic code for pyoderma gangrenosum in an electronic database

Lockwood, Stephen J.; Li, D.G.; Butler, David F.; Tsiaras, William G.; Joyce, Cara; Mostaghimi, Arash

doi:10.1111/bjd.16446

Cited by 14 publications

(6 citation statements)

References 8 publications

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“…The main strength of this study is the independent review by two expert investigators to determine the final study cohort, as it has been recently suggested that the reliability of the International Classification of Diseases (ICD) code alone for PG diagnosis is poor (Lockwood et al, 2018). We also assessed the diagnostic framework against a population of patients seen at general dermatology clinics and during inpatient hospital admission.…”

Section: Discussionmentioning

confidence: 99%

Comparison of Three Diagnostic Frameworks for Pyoderma Gangrenosum

Haag

Hansen

Hajar

et al. 2021

Journal of Investigative Dermatology

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Pyoderma gangrenosum (PG) is an inflammatory condition characterized by chronic cutaneous ulcerations. There are three proposed PG diagnostic frameworks (Su, PARACELSUS, Delphi); however, they lack consensus, and their performance has not yet been validated in a well-defined cohort of patients with PG. In this crosssectional retrospective cohort study, we sought to evaluate and compare the concordance of these diagnostic frameworks within a single-institution cohort of patients with PG. There were 47 patients from an initial 76 identified by International Classification of Diseases-9 and/or International Classification of Diseases-10 codes, where two PG experts agreed in their diagnosis of PG on the basis of clinical descriptions, photographs, and pathology. This group was the PG cohort by which we evaluated the performance and concordance of the diagnostic frameworks. The PARACELSUS score identified the highest proportion of patients with PG (89% [42 of 47 patients]), followed by Delphi and Su criteria, each at 74% (35 of 47 patients). Assessment of multirater agreement found that the three criteria agreed in their diagnoses for 72% of the patients (95% confidence interval ¼ 60e85%); chance-adjusted agreement was determined to be 0.44 (95% confidence ¼ 0.16e0.68, Fleiss' kappa). Future research should seek to refine these diagnostic frameworks and identify targeted methods of testing to reduce rates of PG misdiagnosis and patient misclassification in clinical trials.

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Section: Discussionmentioning

confidence: 99%

Comparison of Three Diagnostic Frameworks for Pyoderma Gangrenosum

Haag

Hansen

Hajar

et al. 2021

Journal of Investigative Dermatology

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“…This study was approved by Mass General Brigham’s and Mount Sinai Hospital’s institutional review boards. By a validated approach, International Classification of Diseases (ICD) codes identified patients with ulcerative PG seen between January 2015 and March 2021 5 . Cases with inadequate documentation or lack of definitive diagnosis by a dermatologist were excluded.…”

Section: Cohort Characteristics Pg Confirmed (N = 127) Pg Misdiagnos...mentioning

confidence: 99%

“…By a validated approach, International Classification of Diseases (ICD) codes identified patients with ulcerative PG seen between January 2015 and March 2021. 5 Cases with inadequate documentation or lack of definitive diagnosis by a dermatologist were excluded. Patients eventually rediagnosed were categorized as misdiagnoses.…”

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confidence: 99%

“…Though further prospective studies are warranted to investigate the role of novel diagnostic criteria and histopathology, our study concurs with a previous study that PARACELSUS might outperform Delphi in a real-world setting. 6 Michelle S. Min iD , 1,2 Kylee Kus, 1,3 Nancy Wei, 4,5 Bina Kassamali, 1 Adam Faletsky, 1,6 Epidermal necrolysis: characterization of different phenotypes using an unsupervised clustering analysis DOI: 10.1111/bjd.21009 DEAR EDITOR, Epidermal necrolysis (EN), including Stevens-Johnson syndrome and toxic epidermal necrolysis, is a rare life-threatening condition, caused by drugs in 85% of cases, with an overall mortality of around 15% in the acute phase. 1,2 Two scores predicting mortality, SCORTEN (a severity-of-illness score) and ABCD-10, include clinical and biological data such as involved body surface area (BSA), age, cancer history and urea level.…”

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confidence: 99%

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Evaluating the role of histopathology in diagnosing pyoderma gangrenosum using Delphi and PARACELSUS criteria: a multicentre, retrospective cohort study

et al. 2022

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“…Our patients' disease was diagnosed by using the Su criteria, and the overall demographics of our cohort are consistent with the known epidemiology of the disease in the United States. 2,3 Our study (Xia et al 4 ), however, was based on data from 2 academic institutions in Boston, Massachusetts, and none of the patients included in the study had associated Behçet disease. This accident of geography reflects the regional differences in the prevalence of Behçet disease, which ranges from 80 to 370 cases per 100,000 individuals in Turkey to between 1 per 15,000 population and 1 per 500,000 population in studies conducted in the United States.…”

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confidence: 99%

Reply to: “Re-evaluating pyoderma gangrenosum patients for Behçet disease before initiating any invasive procedures is essential”

Mostaghimi

Joyce

2018

Journal of the American Academy of Dermatology

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must consider the representativeness of the study sample when we generalize results to other settings or populations. Our patients' disease was diagnosed by using the Su criteria, and the overall demographics of our cohort are consistent with the known epidemiology of the disease in the United States. 2,3 Our study (Xia et al 4 ), however, was based on data from 2 academic institutions in Boston, Massachusetts, and none of the patients included in the study had associated Behçet disease. This accident of geography reflects the regional differences in the prevalence of Behçet disease, which ranges from 80 to 370 cases per 100,000 individuals in Turkey to between 1 per 15,000 population and 1 per 500,000 population in studies conducted in the United States. 5 A similar study performed in areas where Behçet disease is endemic would likely represent a different patient population and might reach different conclusions.Our hope is that dermatology research matures to the point at which large, multi-institutional, multinational prospective trials are the backbone of robust research networks that inform clinicians and researchers with a level of rigor and generalizability that will never be achieved in retrospective regional studies such as ours. To realize this future, we must persuade researchers to relinquish some of their control and share authorship, encourage institutions to share data more readily, and convince funding agencies to support investigation into rare diseases.We retain optimism that with hard work and concentrated effort, the better angels of our nature will prevail and lead to a golden age of dermatology investigation. Until then, articles such as ours can be considered amuse-bouche that inspire a feast of greater insights to come.

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The validity of the diagnostic code for pyoderma gangrenosum in an electronic database

Cited by 14 publications

References 8 publications

Comparison of Three Diagnostic Frameworks for Pyoderma Gangrenosum

Comparison of Three Diagnostic Frameworks for Pyoderma Gangrenosum

Evaluating the role of histopathology in diagnosing pyoderma gangrenosum using Delphi and PARACELSUS criteria: a multicentre, retrospective cohort study

Reply to: “Re-evaluating pyoderma gangrenosum patients for Behçet disease before initiating any invasive procedures is essential”

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