What is the management of skin-limited leukocytoclastic vasculitis (LCV)?

Management of Skin-Limited Leukocytoclastic Vasculitis

For skin-limited leukocytoclastic vasculitis (LCV), the recommended first-line treatment is conservative management with symptomatic measures, including leg elevation, compression stockings, and avoidance of triggers, with colchicine or dapsone as first-line pharmacologic options for persistent or recurrent cases. 1, 2, 3

Initial Assessment and Diagnosis

Before initiating treatment, confirm that the vasculitis is truly skin-limited:

• Obtain skin biopsy with immunofluorescence to confirm diagnosis and identify immunoglobulin type (IgA vs IgG/IgM)
• Complete basic workup including CBC, renal function tests, urinalysis
• Screen for potential triggers (medications, infections, malignancy)
• Rule out systemic involvement through appropriate testing

Treatment Algorithm for Skin-Limited LCV

Step 1: Conservative Management (First-Line)

• Leg elevation when affected areas are on lower extremities
• Compression stockings to reduce purpura
• Avoidance of prolonged standing
• Protection from cold temperatures
• Avoidance of identified triggers
• NSAIDs or antihistamines for symptomatic relief

Step 2: Mild Persistent or Recurrent Disease

• Colchicine (first-line pharmacologic therapy) 1, 2
• Dapsone (second-line if colchicine fails) 1, 2, 3

Step 3: Progressive Disease with Skin Necrosis or Ulceration

• Oral corticosteroids (prednisone 0.5-1 mg/kg/day) with tapering schedule 1, 2
• Target reduction to 15-20 mg/day within 2-3 months
• Aim for ≤5 mg/day after 1 year

Step 4: Severe Recalcitrant Cutaneous Disease

• Consider immunosuppressive agents:
  • Methotrexate
  • Azathioprine
  • Mycophenolate mofetil 3, 4

Special Considerations

IgA vs IgG/IgM Predominance

• IgA-dominant LCV (Henoch-Schönlein purpura) has higher risk of systemic involvement and requires closer monitoring 2, 5
• IgG/IgM-dominant LCV typically has better prognosis and less systemic involvement 2

Monitoring

• Follow-up every 1-3 months during first year of treatment
• Monitor for disease activity and treatment-related adverse effects
• For patients on immunosuppressants, follow appropriate monitoring protocols:
  • CBC and liver function tests for methotrexate
  • CBC for azathioprine
  • G6PD levels before initiating dapsone

Treatment Pitfalls to Avoid

1. Overtreatment: Most cases of skin-limited LCV are self-limited and do not require aggressive immunosuppression
2. Undertreatment: Failing to escalate therapy when signs of skin necrosis or ulceration develop
3. Missing systemic disease: Inadequate screening for systemic involvement before labeling as "skin-limited"
4. Prolonged steroid use: Extended high-dose corticosteroid therapy without appropriate tapering or steroid-sparing agents

When to Consider Referral

• Progressive disease despite appropriate therapy
• Uncertainty about skin-limited vs systemic disease
• Development of new symptoms suggesting systemic involvement
• Need for more aggressive immunosuppressive therapy

By following this structured approach to skin-limited LCV, most patients can achieve good disease control with minimal medication-related adverse effects.