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American Journal of Clinical Dermatology

, Volume 18, Issue 3, pp 355–372 | Cite as

Pyoderma Gangrenosum: An Update on Pathophysiology, Diagnosis and Treatment

  • Afsaneh AlaviEmail author
  • Lars E. French
  • Mark D. Davis
  • Alain Brassard
  • Robert S. Kirsner
Review Article

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.

Keywords

Inflammatory Bowel Disease Infliximab Etanercept Adalimumab Anakinra 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Acknowledgements

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

Compliance with Ethical Standards

Funding

No funding was received for the preparation of this review and the paper has not been presented elsewhere.

Conflict of interest

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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Copyright information

© Springer International Publishing Switzerland 2017

Authors and Affiliations

  • Afsaneh Alavi
    • 1
    Email author
  • Lars E. French
    • 2
  • Mark D. Davis
    • 3
  • Alain Brassard
    • 4
  • Robert S. Kirsner
    • 5
  1. 1.Division of Dermatology, Department of MedicineWomen’s College Hospital, University of TorontoTorontoCanada
  2. 2.Department of DermatologyUniversity Hospital of ZurichZurichSwitzerland
  3. 3.Department of DermatologyMayo ClinicRochesterUSA
  4. 4.Department of DermatologyUniversity of AlbertaEdmontonCanada
  5. 5.Department of DermatologyUniversity of Miami Miller School of MedicineMiamiUSA

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