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Updated: Dec 17 2021

Pyoderma Gangrenosum

  • Snapshot
    • A 40-year-old woman with a past medical history of rheumatoid arthritis presents to the inpatient dermatology consult service with 3 ulcers on her abdomen. A few days ago, she underwent a laparoscopic cholecystectomy and had been recovering in the hospital. However, while changing her dressing, the team noticed that she had 3 growing ulcers at the sites of incisions. On physical exam, she has 3 sharply demarcated ulcers with violaceous raised borders, all on a purulent base. Concerned for an inflammatory process, the dermatology team prescribes systemic steroids.
  • Introduction
    • Clinical definition
      • necrotizing noninfectious and inflammatory disease with painful necrotic ulcers
    • Associated conditions
      • more than half of patients have inflammatory diseases
      • inflammatory bowel disease (most common)
      • rheumatoid arthritis
      • seronegative spondyloarthropathies
      • myeloproliferative diseases
  • Epidemiology
    • incidence
      • rare
    • demographics
      • common in patients 20-60 years of age
      • rare in children
    • Pathogenesis
      • due to immune dysregulation
      • inciting event may be due to trauma
  • Presentation
    • Symptoms
      • pathergy (enlargening or worsening lesion) with trauma is characteristic
      • painful lesions on lower legs, buttocks, and abdomen
    • Physical exam
      • multiple tender red pustules that ulcerate
        • sharply demarcated ulcer with raised and violaceous border
        • purulent base
      • fully evolved lesion < 10 cm in diameter
      • lesions may coalesce into larger ulcers with crater-hole lesions
  • Studies
    • Skin biopsy
      • not always diagnostic
      • useful in ruling out other lesions such as vasculitis
      • neutrophilic infiltrate
  • Differential
    • Infected ulcer
    • Behçet disease
    • Wegener’s granulomatosis
    • Spider bite
  • Treatment
    • Medical
      • topical therapy
        • indications
          • superficial lesions
          • localized disease
        • drugs
          • corticosteroids
          • tacrolimus
          • cyclosporine
      • systemic therapy
        • indications
          • deep or large lesions
          • widespread disease
        • drugs
          • steroids
          • cyclosporine
  • Complications
    • Secondary bacterial infection
    • Atrophic scars after healing
  • Prognosis
    • Chronic, relapsing
    • Ulcers leave atrophic scars when healed
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