Updated: 10/30/2016

Pyoderma Gangrenosum

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Snapshot
  • A 35-year-old man with a history of Crohn’s disease presents to his doctor with a large ulcer on his left shin. A week ago, he accidentally ran into the corner of his coffee table and hurt his left shin. Since then, a small pustule that formed at the site after the bump has progressed into a large 5 cm ulcer. On exam, the border is purple and raised. The lesion is extremely painful.
Introduction
  • Necrotizing noninfectious, inflammatory disease with painful necrotic ulcers
  • Pathogenesis
    • due to immune dysregulation
    • inciting event may be due to trauma
  • Epidemiology
    • rare
    • common in patients 20 - 60 year of age
    • rare in children
  • Associated conditions
    • more than half of patients have inflammatory diseases
      • inflammatory bowel disease (most common)
      • rheumatoid arthritis
      • seronegative spondyloarthropathies
    • myeloproliferative diseases
Presentation
  • Symptoms
    • pathergy (enlargening or worsening lesion) with trauma is characteristic
    • painful lesions on lower legs, buttocks, abdomen
  • Physical exam
    • multiple tender red pustules that ulcerate
      • sharply demarcated ulcer with raised, violaceous border
      • purulent base
    • fully evolved lesion < 10 cm in diameter
    • lesions may coalesce into larger ulcers with crater-hole lesions
Evaluation
  • Skin biopsy
    • not always diagnostic
    • useful in ruling out other lesions, like vasculitis
    • neutrophilic infiltrate
Differential
  • Infected ulcer
  • Behçet’s disease
  • Wegener’s granulomatosis
  • Spider bite
Treatment
  • Treat underlying condition
  • For superficial lesions
    • topical corticosteroids, tacrolimus, or cyclosporine
  • For deep/large lesions
    • systemic steroids
    • systemic cyclosporine
Prognosis, Prevention, and Complications
  • Prognosis
    • chronic, relapsing
    • ulcers leave atrophic scars when healed
 

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