What is the recommended treatment for a vasculitic rash?

Treatment of Vasculitic Rash

For vasculitic rash treatment, a combination of glucocorticoids with either cyclophosphamide or rituximab is recommended as first-line therapy, with the specific choice depending on disease severity and organ involvement. 1, 2

Treatment Algorithm Based on Disease Severity

Non-Organ Threatening Disease (Isolated Cutaneous Vasculitis)

• First-line options:
  • Glucocorticoids + Methotrexate 1, 2
  • For mild disease: Colchicine or dapsone 3, 4
  • General measures: Leg elevation, avoiding prolonged standing, avoiding tight clothing 3, 4

Organ or Life-Threatening Disease

• First-line options:
  • Glucocorticoids + Cyclophosphamide 1
    • Oral cyclophosphamide: 2 mg/kg/day (maximum 200 mg/day)
    • OR IV pulse cyclophosphamide (preferred to reduce cumulative dose)
  • Glucocorticoids + Rituximab 1, 2
    • Particularly effective for ANCA-associated vasculitis

Glucocorticoid Dosing

• Initial treatment: Prednisone 1 mg/kg/day (maximum 80 mg/day) 1
• For severe disease: Consider IV methylprednisolone 500-1000 mg/day for 3-5 days before oral prednisone 2
• Taper according to clinical response, aiming for ≤10 mg/day during remission 2

Maintenance Therapy (After Remission)

• Recommended options:
  • Azathioprine (1.5-2 mg/kg/day) 1, 2
  • Methotrexate (20-25 mg/week if creatinine <130 μmol/L) 1, 2
  • Rituximab (500 mg IV every 6 months) 2
• Duration: Minimum 18 months, typically 2-4 years 2
• Continue low-dose glucocorticoids (5-7.5 mg/day) for approximately 2 years 2

Important Considerations

Infection Prevention

• Trimethoprim/sulfamethoxazole prophylaxis for patients on cyclophosphamide 2
  • 800/160 mg on alternate days or 400/80 mg daily

Cyclophosphamide Administration

• Administer antiemetic therapy routinely 1
• Encourage fluid intake or provide IV fluids on infusion day 1
• Consider MESNA to prevent hemorrhagic cystitis 1

Monitoring

• Regular assessment of:
  • Clinical response (improvement in rash)
  • Complete blood count
  • Renal function
  • Electrolytes
  • Blood pressure 2

Treatment for Refractory Cases

• Re-evaluate diagnosis 1
• Optimize current treatment 1
• Consider alternative agents:
  • Mycophenolate mofetil 1, 4
  • Intravenous immunoglobulin 3, 4
  • Plasmapheresis (particularly for severe disease) 3, 4
  • Newer biologics (TNF inhibitors, tocilizumab) 2, 3

Special Considerations

• Fertility preservation counseling before initiating cyclophosphamide 2
• Bone protection for patients on long-term glucocorticoids 2
• Cardiovascular risk management 2
• Referral to centers with experience in vasculitis management for complex cases 2

The treatment approach should be guided by the severity of the vasculitic rash, presence of systemic involvement, and underlying cause of the vasculitis. Early aggressive treatment is essential for organ or life-threatening disease to prevent permanent damage and reduce mortality.