Pyoderma Gangrenosum: An Updated Literature Review on Established and Emerging Pharmacological Treatments

Carlo Alberto Maronese, Matthew A. Pimentel, May M. Li, Giovanni Genovese, Alex G. Ortega-Loayza, Angelo Valerio Marzano

Research output: Contribution to journalReview articlepeer-review

50 Scopus citations

Abstract

Pyoderma gangrenosum is a rare inflammatory skin disease classified within the group of neutrophilic dermatoses and clinically characterized by painful, rapidly evolving cutaneous ulcers with undermined, irregular, erythematous-violaceous edges. Pyoderma gangrenosum pathogenesis is complex and involves a profound dysregulation of components of both innate and adaptive immunity in genetically predisposed individuals, with the follicular unit increasingly recognized as the putative initial target. T helper 17/T helper 1-skewed inflammation and exaggerated inflammasome activation lead to a dysregulated neutrophil-dominant milieu with high levels of tumor necrosis factor-α, interleukin (IL)-1β, IL-1α, IL-8, IL-12, IL-15, IL-17, IL-23, and IL-36. Low-evidence studies and a lack of validated diagnostic and response criteria have hindered the discovery and validation of new effective treatments for pyoderma gangrenosum. We review established and emerging treatments for pyoderma gangrenosum. A therapeutic algorithm based on available evidence is also provided. For emerging treatments, we review target molecules and their role in the pathogenesis of pyoderma gangrenosum.

Original languageEnglish (US)
Pages (from-to)615-634
Number of pages20
JournalAmerican Journal of Clinical Dermatology
Volume23
Issue number5
DOIs
StatePublished - Sep 2022

ASJC Scopus subject areas

  • Dermatology

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