What is the treatment for leucocytoclastic vasculitis?

Treatment of Leucocytoclastic Vasculitis

Critical First Step: Distinguish Isolated Cutaneous from Systemic Disease

The treatment of leucocytoclastic vasculitis fundamentally depends on whether the disease is isolated to the skin or represents cutaneous manifestations of systemic vasculitis—these require entirely different therapeutic approaches. 1

Initial Disease Stratification

• Isolated cutaneous leucocytoclastic vasculitis (skin-limited disease without organ-threatening features) is managed conservatively and does not require aggressive immunosuppression 1, 2
• Systemic vasculitis with cutaneous involvement (life- or organ-threatening manifestations, gangrene, or severe organ involvement) demands immediate high-dose immunosuppression 1, 3
• The ACR/Vasculitis Foundation explicitly excludes isolated cutaneous vasculitis from systemic polyarteritis nodosa treatment protocols 1

Treatment Algorithm for Isolated Cutaneous Leucocytoclastic Vasculitis

First-Line Conservative Management

• Rest and compression stockings to reduce purpura and prevent progression 4
• Identify and eliminate triggering factors: discontinue suspected medications, treat underlying infections (throat swabs, hepatitis B/C testing) 4, 2
• Low-dose corticosteroids (prednisone 0.5 mg/kg/day or less) for symptomatic relief or signs of incipient skin necrosis 4, 2

Second-Line Therapy for Chronic or Relapsing Disease

• Colchicine is recommended as first-line therapy for chronic or relapsing cutaneous leucocytoclastic vasculitis 4, 5
• Dapsone is recommended as second-line therapy when colchicine fails 4, 5
• Azathioprine (1-2 mg/kg/day) has demonstrated effectiveness in chronic cutaneous vasculitis, with 57-75% improvement rates in case series 3, 5

Common Pitfall: Nonsteroidal anti-inflammatory agents, antihistamines, and antimalarials are effective only in sporadic cases and should not be relied upon as primary therapy 5

Treatment Algorithm for Systemic Vasculitis with Cutaneous Manifestations

Severe Disease (Gangrene, Organ-Threatening Features)

Immediate high-dose glucocorticoids combined with cyclophosphamide or rituximab is the standard of care for severe systemic vasculitis with cutaneous involvement. 1, 3

Remission Induction Regimen

• Prednisone 1 mg/kg/day (maximum 80 mg/day) or pulse IV methylprednisolone 1, 3
• Plus cyclophosphamide: either oral 2 mg/kg/day (maximum 200 mg/day) or IV pulse 0.6 g/m² every 2-4 weeks 3
• Or rituximab: 375 mg/m² weekly for 4 weeks (particularly for relapsing disease, with 91% remission rate in refractory ANCA-associated vasculitis) 3, 1

Mandatory Prophylaxis

• Pneumocystis jirovecii prophylaxis: trimethoprim-sulfamethoxazole 800/160 mg on alternate days or 400/80 mg daily for all patients receiving cyclophosphamide 3, 1
• Bone protection: calcium, vitamin D, and bisphosphonates for all patients on glucocorticoids 1
• Uroprotection: MESNA (2-mercaptoethanesulfonate sodium) with cyclophosphamide to prevent hemorrhagic cystitis 3

Glucocorticoid Tapering Strategy

• Maintain high-dose glucocorticoids for minimum 1 month before initiating taper 1
• Target dose: 15-20 mg/day within 2-3 months, then ≤5 mg/day after 1 year 3
• Never taper prematurely—this is a common cause of relapse 1

Maintenance Therapy After Remission Induction

• Azathioprine 1-2 mg/kg/day is the standard maintenance agent after cyclophosphamide-induced remission 3, 1
• Continue maintenance therapy for at least 18 months in complete remission 1
• Alternative maintenance agents: methotrexate or mycophenolate mofetil if azathioprine is contraindicated 3

Critical Monitoring: Check TPMT activity before starting azathioprine—86% of patients with two variant TPMT alleles develop myelosuppression 3

Treatment of Refractory or Relapsing Disease

When Standard Therapy Fails

• Intravenous immunoglobulin (IVIG): 2 g/kg over 5 days for persistent low activity disease 3, 1, 6
• Mycophenolate mofetil: 2 g/day for refractory cases 3, 1
• Rituximab: 375 mg/m² weekly for 4 weeks achieves 91% remission in refractory ANCA-associated vasculitis 3, 1

Important Consideration: IVIG requires pre-treatment measurement of serum immunoglobulin levels—patients with selective IgA deficiency may develop anaphylaxis, and pre-existing hypergammaglobulinemia may lead to hyperviscosity 3

Special Considerations by Etiology

Drug-Induced Leucocytoclastic Vasculitis

• Discontinuation of the culprit drug is usually resolutive with favorable prognosis 2
• Supportive care with rest and compression stockings is typically sufficient 4

Cryoglobulinemic Vasculitis

• Hepatitis C-associated: antiviral therapy is the primary treatment 3
• Essential (non-viral) cryoglobulinemic vasculitis: requires immunosuppressive therapy in collaboration with hepatology 3
• IVIG has demonstrated effectiveness with decrease in cryoglobulin and circulating immune complexes 6

IgA Vasculitis (Henoch-Schönlein Purpura)

• Systemic corticosteroids may reduce severe renal insufficiency in children according to some studies, though evidence in adults is lacking 3, 4
• There is insufficient evidence to support azathioprine for HSP nephritis 3

Monitoring Requirements

• Urinalysis at every visit to screen for renal involvement, infection, or bladder complications (especially with cyclophosphamide exposure) 3
• Complete blood count and renal function every 1-3 months to monitor for drug toxicity and disease activity 3
• Persistent unexplained hematuria in patients with prior cyclophosphamide exposure requires urgent urology referral for transitional cell cancer screening 3
• Serum immunoglobulin levels before each rituximab course and in patients with recurrent infections 3