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Updated: Oct 17 2017

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 
  • 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
  • Associated conditions
    • more than half of patients have inflammatory diseases
    • inflammatory bowel disease (most common)
    • rheumatoid arthritis
    • seronegative spondyloarthropathies
    • myeloproliferative diseases
  • Prognosis
    • chronic, relapsing
    • ulcers leave atrophic scars when healed
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
Private Note

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