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Updated: Oct 30 2016

Pyoderma Gangrenosum

  • 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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