What is the treatment for cutaneous vasculitis?

Treatment for Cutaneous Vasculitis

For cutaneous vasculitis, first-line treatment includes topical steroids for localized disease, while systemic therapy with colchicine or dapsone is recommended for mild recurrent or persistent disease, with more potent immunosuppression (glucocorticoids plus cyclophosphamide or rituximab) reserved for severe cases. 1, 2

Classification and Assessment

Cutaneous vasculitis can present as:

• Palpable purpura or infiltrated erythema (most common)
• Urticaria
• Hemorrhagic vesicles
• Nodules
• Livedo reticularis
• Ulcers
• Digital gangrene

Before initiating treatment, it's essential to determine if the vasculitis is:

• Primary (idiopathic)
• Secondary to underlying conditions (connective tissue diseases, infections, drug reactions)
• Part of a systemic vasculitis syndrome

Treatment Algorithm Based on Disease Severity

1. Mild, Self-Limited Disease

Most cases of cutaneous vasculitis represent a self-limited condition that resolves within 3-4 weeks 3. For these cases:

• General measures:

  • Leg elevation
  • Avoiding prolonged standing
  • Avoiding cold temperatures
  • Avoiding tight-fitting clothing
  • Warming affected areas

• Symptomatic therapy:

  • Antihistamines
  • Aspirin
  • NSAIDs

2. Mild Recurrent or Persistent Disease

For patients with mild but recurrent or persistent cutaneous vasculitis:

• First-line agents:

  • Colchicine
  • Dapsone

• Alternative options:

  • Hydroxychloroquine
  • Azathioprine

3. Severe Cutaneous Disease

For patients with severe, painful, or ulcerative cutaneous lesions:

• Initial therapy:

  • Systemic glucocorticoids (short course)
  • Consider adding steroid-sparing agents early

• Steroid-sparing options:

  • Azathioprine
  • Methotrexate
  • Mycophenolate mofetil

4. Cutaneous Vasculitis with Systemic Involvement

When cutaneous vasculitis is part of a systemic vasculitic syndrome:

• Combination therapy:

  • Glucocorticoids plus cyclophosphamide or rituximab 4
  • For ANCA-associated vasculitis with cutaneous involvement, rituximab 375 mg/m² once weekly for 4 weeks is recommended 5

• Maintenance therapy:

  • Azathioprine (1.5-2 mg/kg/day)
  • Methotrexate (20-25 mg/week if creatinine <130 μmol/L)
  • Rituximab (500 mg IV every 6 months) 6

5. Refractory Cases

For patients who fail standard therapy:

• Advanced options:
  • Plasmapheresis
  • Intravenous immunoglobulin
  • Biologic agents (infliximab, rituximab) 1, 2

Special Considerations

• Hepatitis C-associated cryoglobulinemic vasculitis: Antiviral therapy is recommended 4
• IgA vasculitis (Henoch-Schönlein purpura): May require different management approach than other forms of cutaneous vasculitis 2
• Cutaneous polyarteritis nodosa: Often requires more aggressive immunosuppression 4

Monitoring and Follow-up

• Regular assessment of clinical response
• Monitor for medication side effects
• Evaluate for development of systemic symptoms
• Adjust therapy based on response and tolerability

Pitfalls and Caveats

1. Don't miss systemic involvement: Always assess for signs of internal organ involvement before assuming disease is limited to the skin 7

2. Avoid prolonged steroid use: While effective for acute management, long-term steroid use carries significant morbidity; transition to steroid-sparing agents when possible 3

3. Consider pseudovasculitis: Rule out thrombotic disorders (e.g., antiphospholipid syndrome) that can mimic cutaneous vasculitis 2

4. Identify and remove triggers: Infections, drugs, and environmental factors can trigger or exacerbate cutaneous vasculitis 1, 2

5. Recognize limitations of evidence: Treatment recommendations for cutaneous vasculitis are largely based on expert opinion and case reports rather than high-quality randomized controlled trials 7