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Pyoderma Gangrenosum: An Update on Pathophysiology, Diagnosis and Treatment

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Abstract

Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic disorder with prototypical clinical presentations. Its pathophysiology is complex and not fully explained. Recent information regarding the genetic basis of PG and the role of auto-inflammation provides a better understanding of the disease and new therapeutic targets. PG equally affects patients of both sexes and of any age. Uncontrolled cutaneous neutrophilic inflammation is the cornerstone in a genetically predisposed individual. Multimodality management is often required to reduce inflammation, optimize wound healing, and treat underlying disease. A gold standard for the management of PG does not exist and high-level evidence is limited. Multiple factors must be taken into account when deciding on the optimum treatment for individual patients: location, number and size of lesion/ulceration(s), extracutaneous involvement, presence of associated disease, cost, and side effects of treatment, as well as patient comorbidities and preferences. Refractory and rapidly progressive cases require early initiation of systemic therapy. Newer targeted therapies represent a promising pathway for the management of PG, and the main focus of this review is the management and evidence supporting the role of new targeted therapies in PG.

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Acknowledgements

The authors would like to thank Delaram Farzanfar for her work on the tables and figures in this article.

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Authors and Affiliations

  1. Division of Dermatology, Department of Medicine, Women’s College Hospital, University of Toronto, 76 Grenville St, 5th Floor, Toronto, ON, M5S 1B2, Canada

    Afsaneh Alavi

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

    Lars E. French

  3. Department of Dermatology, Mayo Clinic, MN, Rochester, USA

    Mark D. Davis

  4. Department of Dermatology, University of Alberta, Edmonton, AB, Canada

    Alain Brassard

  5. Department of Dermatology, University of Miami Miller School of Medicine, Miami, FL, USA

    Robert S. Kirsner

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Correspondence to Afsaneh Alavi.

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Afsaneh Alavi has been a consultant and investigator for Abbvie, Galderma, Janssen, Novartis, Regeneron, Valeant, and Xoma, and received a grant from Abbvie; Lars E. French received study funding from Novartis; Alain Brassard has been a consultant and speaker for AbbVie, Janssen, Celgene, 3M, and Coloplast; and Robert S. Kirsner has been a consultant for Abbvie. Mark Davis has no conflicts of interest to declare.

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Alavi, A., French, L.E., Davis, M.D. et al. Pyoderma Gangrenosum: An Update on Pathophysiology, Diagnosis and Treatment. Am J Clin Dermatol 18, 355–372 (2017). https://doi.org/10.1007/s40257-017-0251-7

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