What is the treatment for Vasculitis leucocitoclastica (hypersensitivity vasculitis)?

Treatment for Leukocytoclastic Vasculitis (Hypersensitivity Vasculitis)

The treatment for leukocytoclastic vasculitis should be tailored based on disease severity, with skin-limited disease typically managed with rest, compression stockings, and low-dose corticosteroids, while systemic disease requires high-dose glucocorticoids (40-60 mg/day prednisone) plus immunosuppressive agents like cyclophosphamide or rituximab. 1

Disease Classification and Assessment

Before initiating treatment, it's crucial to determine whether the vasculitis is:

1. Skin-limited LCV: Confined to cutaneous manifestations
2. Systemic LCV: With internal organ involvement
3. Secondary LCV: Due to underlying causes (medications, infections, malignancy)

Essential diagnostic workup includes:

• Skin biopsy with immunofluorescence
• Complete blood count
• Renal function tests and urinalysis
• Serological tests (ANCA, ANA, complement levels)
• Evaluation for potential triggers (medications, infections, malignancy) 1

Treatment Algorithm

1. Skin-Limited Leukocytoclastic Vasculitis

• First-line management:

  • Identify and remove triggering factors (medications, infections)
  • Rest and elevation of affected limbs
  • Compression stockings to reduce purpura
  • Low-dose corticosteroids (if needed for symptom control)

• For chronic or relapsing skin-limited LCV:

  • Colchicine as first-line therapy
  • Dapsone as second-line therapy 2

2. Systemic Leukocytoclastic Vasculitis

• For organ-threatening or life-threatening disease:

  • High-dose glucocorticoids (40-60 mg/day prednisone equivalent)
  • PLUS either:
    • Cyclophosphamide (oral: 2 mg/kg/day, max 200 mg/day; OR intravenous: 0.6 mg/m² initially every 2 weeks for a month, then every 4 weeks)
    • Rituximab (375 mg/m² once weekly for four infusions) 1

• For less severe systemic disease:

  • Methotrexate or mycophenolate mofetil may be used instead of cyclophosphamide/rituximab 1

• For rapidly progressive renal failure (serum creatinine >300 µmol/L):

  • Consider plasma exchange 1

3. Secondary Leukocytoclastic Vasculitis

• Treatment of the underlying cause is essential:
  • Discontinuation of culprit medications
  • Treatment of infections
  • Management of associated malignancy 3

Maintenance Therapy

After achieving remission:

• Continue maintenance therapy with azathioprine or methotrexate for at least 24 months
• Consider rituximab for maintenance in selected cases
• Taper high-dose corticosteroids to 15-20 mg/day within 2-3 months, and to ≤5 mg/day after 1 year 1

Prophylaxis and Monitoring

• Pneumocystis jirovecii pneumonia prophylaxis:

  • Trimethoprim-sulfamethoxazole (800/160 mg on alternate days or 400/80 mg daily) for patients on cyclophosphamide, rituximab, or high-dose glucocorticoids 1

• Monitoring:

  • Regular blood counts for patients on cyclophosphamide
  • Serum immunoglobulin concentrations prior to each course of rituximab
  • Urinalysis (risk of hemorrhagic cystitis with cyclophosphamide)
  • Consider mesna for bladder protection with cyclophosphamide 1

• Follow-up schedule:

  • Every 1-3 months during the first year
  • Every 3-6 months thereafter 1

Special Considerations

• Most cases of hypersensitivity vasculitis are self-limited if the precipitant can be removed 4
• Systemic involvement in hypersensitivity vasculitis is rare, and prognosis is generally excellent when limited to the skin 4
• When IgA is the dominant immunoglobulin in immune complexes (Henoch-Schönlein purpura), systemic involvement is more likely in both children and adults 2
• Vascular surgery consultation is indicated for critical limb ischemia, arterial vessel dissection, or aneurysm formation 1

Remember that early diagnosis and appropriate treatment based on disease severity are essential to prevent complications and improve outcomes in patients with leukocytoclastic vasculitis.