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Underlying Systemic Diseases in Pyoderma Gangrenosum: A Systematic Review and Meta-Analysis

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Abstract

Background

There is little consensus regarding the prevalence and distribution of underlying systemic diseases among patients with pyoderma gangrenosum.

Objective

The objective of this study was to synthesize existing data on the prevalence of associated systemic diseases in patients with pyoderma gangrenosum.

Methods

We performed a systematic review and meta-analysis of observational studies in MEDLINE, EMBASE, and Scopus (1823–2017). The quality of evidence was assessed using a modified Newcastle–Ottawa Scale. A meta-analysis was performed using random-effects models to estimate pooled prevalence rates with 95% confidence intervals.

Results

Twenty-one eligible studies comprising 2611 patients with pyoderma gangrenosum were included in the quantitative synthesis. The overall random-effects pooled prevalence of associated systemic diseases was 56.8% (95% confidence interval 45.5–67.4). The leading underlying disease was inflammatory bowel disease (17.6%; 95% confidence interval 13.0–22.7), followed by arthritis (12.8%; 95% confidence interval 9.2–16.9), hematological malignancies (8.9%; 95% confidence interval 6.5–11.6), and solid malignancies (7.4%; 95% confidence interval 5.8–9.1). In 16.3% (95% confidence interval 7.7–27.1) of cases, the onset of pyoderma gangrenosum was attributed to the pathergy phenomenon.

Conclusions

More than half of patients with pyoderma gangrenosum present with a relevant underlying disease. Inflammatory bowel disease and arthritis are the most frequently associated diseases. Relative to the reported literature, the pooled prevalence of arthritis and hematological malignancies is lower, while the pooled prevalence of solid malignancies is higher. Owing to the high level of heterogeneity among most of the comparisons, results should be interpreted with caution.

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Author information

Authors and Affiliations

  1. Department of Dermatology, Rambam Health Care Campus, PO Box 9602, Haifa, 31096, Israel

    Khalaf Kridin

  2. Department of Quality Measurements and Research, Chief Physician’s Office, Clalit Health Services, Tel Aviv, Israel

    Arnon D. Cohen

  3. Faculty of Health Sciences, Siaal Research Center for Family Medicine and Primary Care, Ben-Gurion University of the Negev, Beer-Sheva, Israel

    Arnon D. Cohen

  4. Department of Dermatology, University of California Irvine Health, Irvine, CA, USA

    Kyle T. Amber

Authors
  1. Khalaf Kridin
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Corresponding author

Correspondence to Khalaf Kridin.

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Funding

No funding was received for the conduct of this study or the preparation of this article.

Conflict of interest

Khalaf Kridin, Arnon D. Cohen, and Kyle T. Amber have no conflicts of interest directly relevant to the content of this article.

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Kridin, K., Cohen, A.D. & Amber, K.T. Underlying Systemic Diseases in Pyoderma Gangrenosum: A Systematic Review and Meta-Analysis. Am J Clin Dermatol 19, 479–487 (2018). https://doi.org/10.1007/s40257-018-0356-7

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  • DOI: https://doi.org/10.1007/s40257-018-0356-7

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