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Pyoderma gangrenosum

Nature Reviews Disease Primers volume 6, Article number: 81 (2020) Cite this article

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Abstract

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that presents with rapidly developing, painful skin ulcers hallmarked by undermined borders and peripheral erythema. Epidemiological studies indicate that the average age of PG onset is in the mid-40s, with an incidence of a few cases per million person-years. PG is often associated with a variety of other immune-mediated diseases, most commonly inflammatory bowel disease and rheumatoid arthritis. The cause of PG is not well understood, but PG is generally considered an autoinflammatory disorder. Studies have focused on the role of T cells, especially at the wound margin; these cells may support the destructive autoinflammatory response by the innate immune system. PG is difficult to diagnose as several differential diagnoses are possible; in addition to clinical examination, laboratory tests of biopsied wound tissue are required for an accurate diagnosis, and new validated diagnostic criteria will facilitate the process. Treatment of PG typically starts with fast-acting immunosuppressive drugs (corticosteroids and/or cyclosporine) to reduce inflammation followed by the addition of more slowly acting immunosuppressive drugs with superior adverse event profiles, including biologics (in particular, anti-tumour necrosis factor (TNF) agents). Appropriate wound care is also essential. Future research should focus on PG-specific outcome measures and PG quality-of-life studies.

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Fig. 1: Monogenic autoinflammatory syndromes affecting IL-1β activation.
Fig. 2: The complex pathophysiology of ulcers in PG.
Fig. 3: Morphological variation in PG lesions.
Fig. 4: International consensus diagnostic criteria for ulcerative PG.
Fig. 5: Proposed algorithm for treatment of PG.

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Acknowledgements

E.G. is supported by the Swiss Cancer Research Foundation (KFS-4243-08-2017), the Promedica Stiftung (1406/M&1412/M) and the Swiss National Science Foundation (PMPDP3_151326), S.M.L. is supported by a Wellcome Senior Research Fellowship in Clinical Science (205039/Z/16/Z). The authors thank S. Chu (University of California, Davis, Sacramento, CA, USA) for help with artwork.

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Authors and Affiliations

  1. Department of Dermatology, University of California, Davis, Sacramento, CA, USA

    Emanual Maverakis & Stephanie T. Le

  2. Dermatology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy

    Angelo V. Marzano

  3. Department of Physiopathology and Transplantation, Università degli Studi di Milano, Milan, Italy

    Angelo V. Marzano

  4. Department of Medicine, Division of Dermatology, University of Louisville, Louisville, KY, USA

    Jeffrey P. Callen

  5. Department of Dermatology, Zurich University Hospital, Zurich, Switzerland

    Marie-Charlotte Brüggen

  6. Faculty of Medicine, University Zurich, Zurich, Switzerland

    Marie-Charlotte Brüggen

  7. Department of Dermatology, Lausanne University Hospital, Lausanne, Switzerland

    Emmanuella Guenova

  8. Faculty of Biology and Medicine, University of Lausanne, Lausanne, Switzerland

    Emmanuella Guenova

  9. Department of Dermatology, Venereology and Allergology, University Medical Center Essen, Essen, Germany

    Joachim Dissemond

  10. Department of Dermatology, University of California, San Francisco, San Francisco, CA, USA

    Kanade Shinkai

  11. Faculty of Epidemiology and Population Health, London School of Hygiene and Tropical Medicine, London, UK

    Sinéad M. Langan

  12. St John’s Institute of Dermatology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

    Sinéad M. Langan

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  1. Emanual Maverakis
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Contributions

Introduction (all authors); Epidemiology (all authors); Mechanisms/pathophysiology (all authors); Diagnosis, screening and prevention (all authors); Management (all authors); Quality of life (all authors); Outlook (all authors); Overview of Primer (E.M.).

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Correspondence to Emanual Maverakis.

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Competing interests

J.P.C. has ownership of trusts that own stock in Amgen, Pfizer, 3M, Johnson & Johnson, Merck, Abbott Laboratories, AbbVie, Procter and Gamble and Allergen. J.P.C. is a member of a safety monitoring committee for Principia Biopharma. All other authors declare no competing interests.

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Nature Reviews Disease Primers thanks A. Alavi, R. Kirsner, T. Yamamoto, A. A. de Jesus and the other, anonymous, reviewer(s) for their contribution to the peer review of this work.

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Maverakis, E., Marzano, A.V., Le, S.T. et al. Pyoderma gangrenosum. Nat Rev Dis Primers 6, 81 (2020). https://doi.org/10.1038/s41572-020-0213-x

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