What is the treatment for leukocytoclastic vasculitis?

Treatment of Leukocytoclastic Vasculitis

The treatment of leukocytoclastic vasculitis (LCV) should be based on disease severity, with skin-limited disease managed conservatively using colchicine, dapsone, or low-dose corticosteroids, while systemic disease requires aggressive immunosuppression with high-dose glucocorticoids plus cyclophosphamide or rituximab. 1

Disease Classification and Assessment

Determining Disease Extent

• First step: Determine if LCV is skin-limited or systemic
• Diagnostic workup should include:
  • Skin biopsy with immunofluorescence (essential for diagnosis)
  • Complete blood count
  • Renal function tests and urinalysis
  • Serological tests for hepatitis B and C
  • Autoantibody testing (ANA, ANCA)
  • Complement levels
  • IgA staining in biopsy specimens 2

Causes to Identify

• Medications (penicillins, cephalosporins, sulfonamides, phenytoin, allopurinol)
• Infections (particularly streptococcal)
• Malignancy
• Systemic diseases (ANCA-associated vasculitis, connective tissue diseases, cryoglobulinemic vasculitis, IgA vasculitis) 2, 3

Treatment Algorithm

1. Skin-Limited LCV

• First-line options:
  • Colchicine (preferred first-line for chronic/relapsing cases) 4
  • Dapsone (second-line therapy for chronic/relapsing cases) 4
  • Low-dose corticosteroids for symptomatic relief
  • Topical dapsone may be considered in selected patients unable to tolerate systemic medications 5
• Supportive measures:
  • Rest (avoiding standing or walking)
  • Compression stockings to reduce purpura 4
• If medication-induced:
  • Discontinuation of culprit drug is usually curative 2

2. Systemic LCV

• For non-organ threatening disease:

  • Combination of glucocorticoids with methotrexate or mycophenolate mofetil 6

• For organ or life-threatening disease:

  • High-dose glucocorticoids (40-60 mg/day prednisone-equivalent) initiated immediately
  • Combined with either cyclophosphamide or rituximab 6, 1

• For severe diffuse alveolar hemorrhage or rapidly progressive glomerulonephritis:

  • Consider plasma exchange 6

3. Remission Maintenance

• Continue therapy for at least 24 months following induction of sustained remission
• Options include:
  • Low-dose glucocorticoids combined with:
    • Azathioprine
    • Rituximab
    • Methotrexate
    • Mycophenolate mofetil 6, 1

4. Refractory Disease

• Switch from cyclophosphamide to rituximab or vice versa
• Refer to specialized centers for evaluation and potential clinical trial enrollment 6, 1

Special Considerations

Monitoring

• Regular clinical assessment rather than ANCA testing should guide treatment decisions
• Monitor for immunoglobulin levels prior to each rituximab course and in patients with recurrent infections
• Investigate persistent unexplained hematuria in patients with prior cyclophosphamide exposure 6

Prognosis

• Skin-limited LCV generally has favorable prognosis
• Systemic vasculitis has variable prognosis depending on organ involvement
• Relapses may occur years after remission, necessitating long-term follow-up 1, 4

Pitfalls and Caveats

• Do not rely solely on ANCA testing to guide treatment decisions
• Do not discontinue maintenance therapy too early (continue for at least 24 months)
• Do not delay aggressive treatment in organ or life-threatening disease
• Be aware that LCV can be the presenting symptom of severe systemic ANCA-associated vasculitis 7
• Corticosteroids alone may not prevent severe renal insufficiency in adults with systemic vasculitis 4

By following this treatment algorithm and working in collaboration with specialists based on organ involvement, patients with LCV can be effectively managed to reduce morbidity and mortality associated with this condition.