What is the treatment for leukocytoclastic vasculitis compatible with urticarial vasculitis?

Treatment for Urticarial Vasculitis

For urticarial vasculitis, corticosteroids are the most effective first-line treatment, especially when there are signs of incipient skin necrosis or systemic involvement. 1

Treatment Algorithm

Initial Assessment

• Determine severity:
  • Skin-limited vs. systemic involvement
  • Normocomplementemic vs. hypocomplementemic (hypocomplementemic cases typically have more severe multi-organ involvement) 2
• Check for underlying causes:
  • Drugs, infections, autoimmune disorders, malignancies 2

Treatment Approach

For Mild, Skin-Limited Disease:

1. General measures:

   • Leg elevation
   • Avoidance of prolonged standing
   • Avoidance of cold temperatures and tight-fitting clothing
   • Compression stockings to reduce purpura 3, 4

2. First-line pharmacologic options:

   • Antihistamines (note: often ineffective for true urticarial vasculitis) 1
   • NSAIDs 3
   • Colchicine (recommended as first-line for chronic/relapsing cases) 4
   • Dapsone (recommended as second-line for chronic/relapsing cases) 4
   • Hydroxychloroquine 2

For Moderate to Severe Disease or Systemic Involvement:

1. Corticosteroids:

   • Indicated when there are signs of incipient skin necrosis 4
   • Effective in >80% of urticarial vasculitis cases 1
   • Required for necrotizing skin lesions or visceral involvement 2

2. Steroid-sparing agents (for corticosteroid-resistant cases or to reduce steroid dependence):

   • Azathioprine
   • Methotrexate
   • Cyclophosphamide
   • Cyclosporine
   • Mycophenolate mofetil 2, 1

3. For refractory cases:

   • Biologics (omalizumab)
   • Intravenous immunoglobulin
   • Plasmapheresis 1, 5

Important Considerations

• Diagnostic confirmation is essential: Clinical features alone may overlap with allergic urticaria; a lesional skin biopsy is required to confirm the diagnosis 2

• Treatment duration: Individualize based on response and underlying cause; some cases may be self-limited while others require long-term management

• Monitoring: Regular assessment for systemic involvement, particularly in hypocomplementemic patients who have higher risk of multi-organ disease 2

• Ineffective treatments: H1-antihistamines, montelukast, danazol, H2-antihistamines, pentoxifylline, doxepin, and tranexamic acid have shown poor efficacy in most urticarial vasculitis patients 1

Pitfalls to Avoid

• Failing to identify and address underlying causes (drugs, infections, autoimmune disorders)
• Inadequate treatment of systemic involvement
• Prolonged corticosteroid use without steroid-sparing agents
• Mistaking urticarial vasculitis for common urticaria (leading to ineffective antihistamine-only treatment)
• Not distinguishing between normocomplementemic and hypocomplementemic forms, which have different prognoses and treatment needs 2

The most recent and comprehensive systematic review (2019) confirms that corticosteroids remain the cornerstone of treatment, with various immunomodulatory agents serving as steroid-sparing options for long-term management 1.