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Internal Medicine

Pyoderma gangrenosum

Uncommon, ulcerative cutaneous condition of uncertain etiology

Uncommon, ulcerative cutaneous condition of uncertain etiology

  • #2 M/C cutaneous manifestation of IBD (1–3%)

Aetiology

Associated conditions:

  • Inflammatory bowel disease (M/C cause):
    • Ulcerative colitis
    • Crohn’s disease
  • Arthritides:
    • Rheumatoid arthritis
    • Seronegative arthritis
  • Hematological disease:
    • Myelocytic leukaemia
    • Hairy cell leukaemia
    • Myelofibrosis
    • Myeloid metaplasia
    • Monoclonal gammopathy
  • Autoinflammatory disease:
    • Pyogenic sterile arthritis, pyoderma gangrenosum, and acne syndrome (PAPA syndrome)

Clinical features

Primary types:

  • Typical (ulcerative) form
    • Mainly in the legs
  • Atypical form
    • More superficial and occurs in the hands and other parts of the body

Other variations:

  • Peristomal pyoderma gangrenosum (15% cases)
  • Bullous pyoderma gangrenosum
  • Pustular pyoderma gangrenosum
  • Vegetative pyoderma gangrenosum

Diagnosis

Diagnostic criteria

Biopsy demonstrating a neutrophilic infiltrate + ≥ 4 minor criteria:.
  • Histology: Exclusion of infection (including histologically indicated stains and tissue cultures)
  • Pathergy (ulcer occurring at sites of trauma, with ulcer extending past area of trauma)
  • Personal history of inflammatory bowel disease or inflammatory arthritis
  • History of papule, pustule, or vesicle that rapidly ulcerated
  • Clinical examination (or photographic evidence) of peripheral erythema, undermining border, and tenderness at site of ulceration
  • Multiple ulcerations (at least 1 occurring on an anterior lower leg)
  • Cribriform or “wrinkled paper” scar(s) at sites of healed ulcers
  • Decrease in ulcer size within 1 mo of initiating immunosuppressive medication(s)

Management

First-line therapy:

  • Systemic treatmentCorticosteroidsciclosporin
  • Topical treatment: Clobetasol/mupirocin/gentamicintacrolimus

If ineffective, alternative therapeutic procedures:

  • Systemic treatment:
    • Corticosteroids + mycophenolate mofetil
    • Mycophenolate mofetil + ciclosporin
    • Tacrolimus
    • Thalidomide
    • Infliximab
    • Plasmapheresis

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