What is the workup and management of leukocytoclastic vasculitis?

Workup and Management of Leukocytoclastic Vasculitis

Leukocytoclastic vasculitis (LCV) requires a comprehensive diagnostic workup to identify underlying causes, followed by treatment with glucocorticoids combined with immunosuppressive agents for organ-threatening disease or symptomatic management for skin-limited disease.

Diagnostic Workup

Clinical Evaluation

• Assess for palpable purpura (primary clinical presentation)
• Document distribution pattern (typically lower extremities)
• Check for signs of systemic involvement:
  • Renal: hematuria, proteinuria
  • Gastrointestinal: abdominal pain, bleeding
  • Joint: arthralgia, arthritis
  • Neurological: neuropathy, CNS involvement

Laboratory Testing

• Complete blood count with differential
• Comprehensive metabolic panel with renal function
• Urinalysis (to detect hematuria/proteinuria)
• Inflammatory markers: ESR, CRP
• Autoantibody testing:
  • ANCA (for ANCA-associated vasculitis)
  • ANA (for connective tissue diseases)
• Complement levels (C3, C4)
• Serum immunoglobulin levels (especially IgA)
• Hepatitis B and C serology
• Cryoglobulins
• Blood cultures if infection suspected

Histopathological Confirmation

• Skin biopsy is essential for diagnosis 1
• Direct immunofluorescence to identify:
  • IgA deposits (suggests IgA vasculitis/Henoch-Schönlein purpura)
  • IgG/IgM deposits (typical of immune complex LCV)
  • Absence of immune deposits (pauci-immune vasculitis)

Additional Testing Based on Clinical Suspicion

• Chest imaging if pulmonary involvement suspected
• Renal biopsy if significant renal involvement
• Throat swabs if streptococcal infection suspected
• Age-appropriate cancer screening if paraneoplastic etiology suspected

Management

Identify and Remove Triggering Factors

• Discontinue potential culprit medications 2, 3
• Treat underlying infections
• Address associated systemic diseases

Skin-Limited LCV

1. First-line therapy:

   • Rest and elevation of affected limbs
   • Compression stockings to reduce purpura
   • Low-dose corticosteroids (prednisone 0.5-1 mg/kg/day) for symptomatic relief

2. For chronic or relapsing disease:

   • Colchicine (0.6 mg 1-2 times daily) as first-line 4, 5
   • Dapsone (50-200 mg daily) as second-line 4, 5

Organ-Threatening or Life-Threatening LCV

1. Induction therapy 1:

   • High-dose glucocorticoids (40-60 mg/day prednisone-equivalent)
   • PLUS one of:
     • Cyclophosphamide (oral 2 mg/kg/day or IV pulse)
     • Rituximab (375 mg/m² weekly for 4 weeks)

2. For rapidly progressive renal disease:

   • Consider adding plasma exchange 1

3. Maintenance therapy 1:

   • Continue for at least 24 months after achieving sustained remission
   • Options include:
     • Low-dose glucocorticoids with:
       • Azathioprine (2 mg/kg/day)
       • Methotrexate (15-25 mg/week)
       • Mycophenolate mofetil (2 g/day)
       • Rituximab (for maintenance)

Special Considerations

• IgA vasculitis (Henoch-Schönlein purpura):

  • Higher risk of systemic involvement
  • May require more aggressive immunosuppression if renal involvement

• ANCA-associated LCV:

  • Requires aggressive immunosuppression similar to other ANCA vasculitides

• Cryoglobulinemic vasculitis:

  • Treat underlying cause (often hepatitis C)
  • Consider plasmapheresis in severe cases 5

Monitoring and Follow-up

• Regular clinical assessment rather than ANCA testing should guide treatment decisions 6
• Monitor for treatment complications:
  • Investigate persistent unexplained hematuria in patients with prior cyclophosphamide exposure 6
  • Test serum immunoglobulin levels prior to each course of rituximab and in patients with recurrent infection 6
• Periodic assessment of cardiovascular risk 6
• Long-term follow-up is necessary as relapses may occur years after remission 6

Referral

• Patients with LCV should be managed in close collaboration with, or at, centers of expertise 6
• Consider referral to specialized centers for refractory disease or for enrollment in clinical trials 6

Prognosis

• Skin-limited LCV generally has favorable prognosis
• Drug-induced LCV typically resolves with discontinuation of the culprit medication 2, 3
• Systemic vasculitis requires more aggressive therapy and has variable prognosis depending on organ involvement