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

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)
JournalAmerican Journal of Clinical Dermatology
DOIs
StateAccepted/In press - 2022

ASJC Scopus subject areas

  • Dermatology

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