What is the best management approach for Vasculitis leucocitoclastica (hypersensitivity vasculitis)?

Management of Leukocytoclastic Vasculitis

The optimal management approach for leukocytoclastic vasculitis (LCV) requires stratification based on disease severity, with skin-limited disease treated with supportive care and mild immunosuppression, while organ-threatening disease requires aggressive immunosuppression with glucocorticoids plus cyclophosphamide or rituximab. 1

Diagnosis and Assessment

• Confirm diagnosis with skin biopsy with immunofluorescence to distinguish between:

  • IgA-associated vasculitis (Henoch-Schönlein purpura) - higher risk of systemic involvement
  • IgG/IgM-associated vasculitis - generally better prognosis 2

• Essential workup:

  • Complete blood count
  • Renal function tests
  • Urinalysis
  • Serological tests (ANCA, ANA, complement levels)
  • Evaluation for potential triggers (medications, infections, malignancy) 1

Treatment Algorithm Based on Disease Severity

1. Skin-Limited LCV (No Organ Involvement)

• First-line:

  • Leg elevation and compression stockings to reduce purpura
  • Avoidance of triggers (excessive standing, drugs, infections)
  • NSAIDs for symptomatic relief 3, 2

• For mild recurrent or persistent disease:

  • Colchicine (first-line)
  • Dapsone (second-line) 3, 2

• For severe cutaneous disease:

  • Systemic corticosteroids (prednisone 0.5-1 mg/kg/day) when there are signs of incipient skin necrosis 2

2. Organ-Threatening or Life-Threatening LCV

• First-line therapy:

  • High-dose glucocorticoids (40-60 mg/day prednisone equivalent) PLUS
  • Either cyclophosphamide OR rituximab 1, 4

• Cyclophosphamide options:

  • Oral: 2 mg/kg/day (maximum 200 mg/day)
  • Intravenous: 0.6 mg/m² initially every 2 weeks for a month, then every 4 weeks 1

• Rituximab dosing:

  • 375 mg/m² of body surface area, once weekly for four infusions 1

• For patients who cannot tolerate cyclophosphamide or rituximab:

  • Methotrexate or mycophenolate mofetil 1

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

  • Consider plasma exchange 4, 1

Maintenance Therapy

• After achieving remission:
  • Continue maintenance therapy with azathioprine or methotrexate for at least 24 months 1
  • Consider rituximab for maintenance in selected cases 1
  • Taper glucocorticoids 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

• Regular monitoring:

  • Blood counts and urinalysis for patients on cyclophosphamide (risk of hemorrhagic cystitis)
  • Consider mesna for bladder protection with cyclophosphamide
  • Monitor serum immunoglobulin concentrations prior to each course of rituximab 1

• Follow-up schedule:

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

Special Considerations

• Vascular surgery consultation is indicated for:

  • Critical limb ischemia
  • Progressive tissue or organ infarction
  • Arterial vessel dissection
  • Aneurysm formation at high risk for rupture 1

• Refractory disease options:

  • Plasmapheresis
  • Intravenous immunoglobulin
  • Biologic therapies (TNF-alpha inhibitors like infliximab) 3

Pitfalls and Caveats

• Do not delay treatment in organ-threatening disease while searching for underlying causes
• Exclude vasculitis-like syndromes (pseudovasculitis) such as thrombotic disorders
• Be aware that cutaneous manifestations may precede systemic involvement
• IgA-dominant immunofluorescence suggests higher risk of systemic involvement and requires closer monitoring
• Delay surgical vascular interventions until disease is quiescent whenever possible 1

The management of LCV requires careful assessment of disease severity and extent of organ involvement to guide appropriate therapy, with the goal of preventing permanent organ damage while minimizing treatment-related complications.