What is the management approach for leukocystoclastic vasculitis?

Management of Leukocytoclastic Vasculitis

The management of leukocytoclastic vasculitis (LCV) requires a stratified approach based on disease severity, with treatment ranging from symptomatic measures for skin-limited disease to immunosuppressive therapy for systemic involvement.

Disease Classification and Assessment

First, determine whether the LCV is:

1. Skin-limited (cutaneous only)
2. Systemic with organ involvement
3. Secondary to an underlying condition

Diagnostic Workup

• Skin biopsy with immunofluorescence (essential for diagnosis and to identify IgA, IgG, or IgM immune complexes)
• Complete blood count
• Renal function tests and urinalysis
• Serological tests:
  • Hepatitis B and C
  • ANCA
  • ANA
  • Complement levels (C3, C4)
• Evaluate for potential triggers:
  • Medications
  • Infections
  • Malignancy

Treatment Algorithm

1. Skin-Limited LCV

For mild disease with palpable purpura only:

• Rest and elevation of affected limbs
• Avoid prolonged standing or walking
• Compression stockings to reduce purpura

For moderate skin-limited disease:

• Low-dose corticosteroids (prednisone 0.5 mg/kg/day) for 1-2 weeks with rapid taper 1
• Colchicine (0.6 mg 1-2 times daily) as first-line steroid-sparing agent 2
• Dapsone (50-100 mg daily) as second-line therapy 2

For chronic/recurrent cutaneous LCV:

• Colchicine or dapsone for maintenance therapy 3
• Consider topical dapsone for patients with contraindications to systemic therapy 4

2. Systemic LCV

For organ-threatening or life-threatening disease:

High-dose glucocorticoids (40-60 mg/day prednisone equivalent) should be initiated immediately for induction of remission, combined with either cyclophosphamide or rituximab 5

For ANCA-associated LCV with systemic involvement:

• Cyclophosphamide (level of evidence 1A for GPA and MPA) or rituximab (level of evidence 1B) 5
• Taper glucocorticoids to 15-20 mg/day within 2-3 months 5
• After 1 year, target ≤5 mg/day of prednisone 5

For IgA vasculitis (Henoch-Schönlein purpura):

• More aggressive treatment needed in adults than children
• High-dose corticosteroids for organ involvement
• Consider additional immunosuppression for severe renal disease

For maintenance therapy after remission:

• Azathioprine or methotrexate 5
• Consider rituximab for relapsing disease 5

3. Management of Secondary LCV

• Medication-induced: Discontinue the offending drug (usually resolves within days to weeks) 1
• Infection-associated: Treat the underlying infection
• Malignancy-associated: Treat the underlying malignancy
• Cryoglobulinemic vasculitis: Treat underlying disease (often hepatitis C or plasma cell disorders) 3

Follow-up and Monitoring

• Regular follow-up every 1-3 months during the first year and in 3-6 month intervals afterward 5
• Monitor for disease activity based on symptoms, clinical findings, and ESR/CRP levels 5
• Screen for treatment-related adverse effects and comorbidities 5

Special Considerations

1. Refractory disease: For patients not responding to standard therapy, consider:

   • Referral to centers with vasculitis expertise 5
   • Alternative immunosuppressants (mycophenolate mofetil, tocilizumab, TNF inhibitors) 5

2. Relapse management:

   • For major relapse: Reinstitute or escalate glucocorticoid therapy as recommended for new-onset disease 5
   • For minor relapse: Increase glucocorticoid dose to at least the last effective dose 5
   • Consider initiation or modification of adjunctive therapy 5

3. Prophylaxis:

   • For patients receiving rituximab, cyclophosphamide and/or high doses of glucocorticoids, use trimethoprim-sulfamethoxazole as prophylaxis against Pneumocystis jirovecii pneumonia 5
   • Monitor serum immunoglobulin concentrations prior to each course of rituximab 5

Prognosis

• Skin-limited LCV generally has a favorable prognosis
• Medication-induced LCV typically resolves with discontinuation of the culprit drug 1
• Systemic vasculitis requires more aggressive therapy and has a variable prognosis depending on organ involvement and underlying disease 1

Remember that LCV management should be conducted in collaboration with, or at, centers of expertise, particularly for cases with systemic involvement 5.