Treatment of Idiopathic Recurrent Cutaneous Vasculitis
For idiopathic recurrent cutaneous vasculitis without organ-threatening features, initiate colchicine or dapsone as first-line therapy, reserving glucocorticoids and immunosuppressants for severe, refractory, or ulcerative disease. 1, 2, 3
Initial Disease Stratification
The critical first step is distinguishing isolated cutaneous vasculitis from systemic disease, as treatment algorithms differ substantially 1:
- Isolated cutaneous disease presents with palpable purpura, infiltrated erythema, or uncomplicated skin lesions without life- or organ-threatening manifestations 1, 4
- Organ-threatening disease includes glomerulonephritis, pulmonary hemorrhage, digital gangrene, or extensive ulceration requiring immediate aggressive immunosuppression 5, 1
The EULAR guidelines explicitly state that isolated cutaneous leukocytoclastic vasculitis without organ-threatening manifestations does not require aggressive immunosuppression 5
Conservative Management for Mild Disease
Most cases of isolated cutaneous vasculitis are self-limited and resolve spontaneously over 3-4 weeks 2:
- Symptomatic measures include leg elevation, avoidance of prolonged standing, warming, and avoidance of cold temperatures and tight-fitting clothing 6, 7
- NSAIDs or aspirin for symptomatic relief 6, 7
- Antihistamines for associated pruritus 6, 7
First-Line Systemic Therapy for Recurrent Disease
For symptomatic, recurrent, extensive, or persistent cutaneous disease 6, 3:
- Colchicine is a first-choice agent for mild recurrent or persistent disease 6, 4, 7, 2
- Dapsone is equally recommended as first-line therapy, used singly or in combination with colchicine 6, 4, 7, 3
- Short-course oral glucocorticoids may be required for painful, ulcerative, or otherwise severe disease to speed resolution 2
Second-Line Options for Refractory Disease
When first-line agents fail or disease remains chronic and recurring 2, 3:
- Azathioprine 1-2 mg/kg/day 1, 4, 3
- Hydroxychloroquine as a reasonable longer-term option 2
- Methotrexate for recurrent chronic disease 4, 3
- Mycophenolate mofetil for refractory cases 4, 7, 3
Severe or Refractory Disease Management
For severely symptomatic cutaneous disease that does not respond to conventional therapies 6, 4:
- Systemic corticosteroids (prednisone 1 mg/kg/day) combined with steroid-sparing agents 1, 6, 4
- Cyclosporine for refractory disease 6, 4, 3
- Rituximab (anti-B-cell antibody) showing benefit in refractory vasculitis 6, 4, 3
- Intravenous immunoglobulin (IVIG) 2 g/kg over 5 days for persistent low activity 1, 6, 4
- Plasmapheresis for refractory cases, though cumbersome and expensive 6, 4, 7
Critical Diagnostic Exclusions
Before initiating treatment, the EULAR guidelines recommend ruling out secondary causes 5:
- Laboratory evaluation including ANCA testing, cryoglobulins, autoantibodies, complement levels, hepatitis B/C serology 5
- Medication review to identify drug-induced vasculitis (propylthiouracil, TNF inhibitors, amiodarone) where discontinuation alone may be sufficient 8
- Exclude pseudovasculitis such as antiphospholipid antibody syndrome or other thrombotic disorders 4
Common Pitfalls
- Avoid aggressive immunosuppression (cyclophosphamide, rituximab, high-dose glucocorticoids) for isolated cutaneous disease without organ involvement, as this represents overtreatment 5, 1
- Do not use cyclophosphamide for isolated cutaneous vasculitis; it is reserved exclusively for organ-threatening systemic disease 1
- Recognize that most cases are self-limited—many patients require no systemic treatment beyond conservative measures 6, 7, 2
- Insufficient evidence exists for routine use of biologic therapies in primary cutaneous vasculitis, though they may be considered in truly refractory cases 7