What is the recommended treatment for cutaneous vasculitis?

Treatment of Cutaneous Vasculitis

For isolated cutaneous vasculitis without systemic involvement, most cases are self-limited and require only conservative management with leg elevation and NSAIDs, reserving systemic therapy for severe, recurrent, or persistent disease. 1

Initial Assessment and Disease Severity Stratification

The first critical step is distinguishing between isolated cutaneous vasculitis and systemic vasculitis with cutaneous manifestations, as treatment differs substantially. 2

Key distinction: The 2021 ACR/Vasculitis Foundation guidelines explicitly exclude isolated cutaneous vasculitis from systemic polyarteritis nodosa treatment protocols, as cutaneous-limited disease is managed differently. 2

Severity Classification

• Nonsevere cutaneous disease: Uncomplicated skin lesions (palpable purpura, infiltrated erythema) without organ-threatening features 2
• Severe cutaneous disease: Extensive ulceration, digital gangrene, or tissue necrosis requiring aggressive intervention 3, 4
• Systemic involvement: Life- or organ-threatening manifestations (glomerulonephritis, mononeuritis multiplex, mesenteric ischemia) demanding immediate immunosuppression 2

Treatment Algorithm for Isolated Cutaneous Vasculitis

Mild, Self-Limited Disease (First-Line)

Most isolated cutaneous vasculitis resolves spontaneously within 3-4 weeks and requires only supportive care. 1

• Conservative measures: Leg elevation, avoidance of prolonged standing, warming, avoidance of cold temperatures and tight-fitting clothing 5, 6
• Symptomatic relief: Antihistamines, aspirin, or NSAIDs for pain and inflammation 5, 7
• Trigger avoidance: Discontinue potential causative medications, treat underlying infections 6

Recurrent or Persistent Disease (Second-Line)

For disease lasting >3 months or with multiple recurrences, escalate to steroid-sparing agents. 1

First-choice steroid-sparing agents:

• Colchicine: Effective in most patients with recurrent disease 5, 7, 1
• Dapsone: First-choice alternative, effective in sporadic to most cases 5, 7, 1

Alternative options for refractory cases:

• Hydroxychloroquine: Reasonable longer-term option 1
• Azathioprine: Effective in most patients, particularly for chronic disease 7, 1

Severe Cutaneous Disease (Third-Line)

For painful, ulcerative, extensive, or otherwise severe isolated cutaneous disease:

• Short-term oral glucocorticoids: Required to speed resolution, typically prednisone 0.5-1 mg/kg/day for limited duration 5, 1
• Combination therapy: Prednisone plus azathioprine, methotrexate, or mycophenolate mofetil for severe disease 5, 6

Important caveat: Avoid prolonged glucocorticoid monotherapy; transition to steroid-sparing agents as soon as feasible. 1

Treatment for Systemic Vasculitis with Cutaneous Involvement

When cutaneous vasculitis represents severe systemic disease (e.g., digital gangrene, extensive ulceration with organ involvement):

Immediate Induction Therapy

The European League Against Rheumatism recommends immediate high-dose glucocorticoids combined with cyclophosphamide (or rituximab) for gangrene or severe organ-threatening cutaneous vasculitis. 3, 4

• High-dose glucocorticoids: Prednisone 1 mg/kg/day (maximum 60-80 mg/day) or IV methylprednisolone 500-1,000 mg/day for 3-5 days 2, 3, 4
• Cyclophosphamide: Combined with glucocorticoids for severe disease 2, 3, 4
• Rituximab: Alternative to cyclophosphamide, particularly for refractory cases (91% remission rate in open-label trials) 2

Maintenance Therapy

• Duration: Maintain high-dose glucocorticoids for minimum 1 month before gradual taper 3, 4
• Transition: Switch to azathioprine 1-2 mg/kg/day for maintenance after remission induction 3, 4
• Duration of maintenance: Continue for at least 18 months in complete remission 3, 4

Adjunctive Therapies

• Pneumocystis prophylaxis: Trimethoprim-sulfamethoxazole (800/160 mg on alternate days or 400/80 mg daily) for all patients receiving cyclophosphamide 3, 4
• Bone protection: Calcium, vitamin D, and bisphosphonates for all patients on glucocorticoids 3, 4
• Plasmapheresis: Consider for rapidly progressive disease or diffuse organ failure 3, 4

Refractory Disease Options

For patients failing standard therapy:

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

Critical Pitfalls to Avoid

• Do not use alternate-day glucocorticoid therapy: Significantly increases relapse risk 3, 4
• Do not overlook systemic involvement: Deep skin biopsy reaching medium-sized vessels is essential to distinguish isolated from systemic disease 2
• Do not use trimethoprim-sulfamethoxazole monotherapy for maintenance: May not be effective alone, though it reduces relapse risk when added to standard therapy 2
• Do not miss pseudovasculitis: Exclude thrombotic disorders (antiphospholipid antibody syndrome) that mimic vasculitis but require anticoagulation, not immunosuppression 6