What is the recommended treatment for a patient with urticaria vasculitis?

Treatment of Urticarial Vasculitis

For urticarial vasculitis, start with high-dose second-generation H1-antihistamines (up to 4 times standard dose), and if inadequate response after 2-4 weeks, add short courses of systemic corticosteroids followed by immunomodulatory agents such as dapsone, hydroxychloroquine, or omalizumab for steroid-sparing effect. 1

Diagnostic Confirmation Before Treatment

• Confirm diagnosis with lesional skin biopsy showing leukocytoclastic vasculitis (leucocytoclasia, endothelial damage, perivascular fibrin deposition, red cell extravasation) if individual wheals persist beyond 24 hours, distinguishing this from ordinary chronic urticaria where lesions last 2-24 hours 2, 1
• Perform full vasculitis screen including serum complement assays to distinguish normocomplementemic from hypocomplementemic disease, as this affects prognosis and treatment intensity 1

Stepwise Treatment Algorithm Based on Disease Severity

Mild Disease (UVAS7 ≤7 of 70, skin-limited symptoms)

• Start with second-generation H1-antihistamines (cetirizine, fexofenadine, loratadine, or desloratadine) at standard doses for 2-4 weeks 1, 3
• Trial at least two different nonsedating antihistamines if first is ineffective, as individual responses vary significantly 1
• Increase antihistamine dose up to 4 times the standard dose if inadequate control after 2-4 weeks, as potential benefits outweigh risks 2, 1
• Add H2-antihistamines or antileukotrienes (montelukast) for resistant cases, though evidence for efficacy is limited 2, 1

Moderate Disease (UVAS7 >7 or persistent symptoms despite high-dose antihistamines)

• Add short tapering courses of oral corticosteroids (prednisolone 0.5-1 mg/kg/day) over 3-4 weeks, as more prolonged treatment may be necessary for urticarial vasculitis compared to ordinary urticaria 2, 1
• Add omalizumab 300 mg subcutaneously every 4 weeks if inadequate response to antihistamines and corticosteroids, allowing up to 6 months for response 1, 4
• Consider updosing omalizumab by shortening interval and/or increasing dosage (maximum 600 mg every 14 days) for insufficient response to standard dosing 1

Severe Disease (UVAS7 >7 with systemic involvement or hypocomplementemic UV)

• Initiate systemic corticosteroids as first-line therapy (prednisolone 0.5-1 mg/kg/day) for rapid control 1
• Add immunomodulatory agents early to allow corticosteroid tapering, as long-term corticosteroids should be avoided except in very selected cases under specialist supervision 1, 4
• First-choice steroid-sparing agents include:
  • Dapsone: Effective for both skin and systemic symptoms in UV, with response rates exceeding 80% when combined with corticosteroids 4, 3
  • Hydroxychloroquine: Effective immunomodulatory option particularly for hypocomplementemic UV 4, 3
  • Cyclosporine A: Use up to 5 mg/kg body weight with monitoring of blood pressure and renal function every 6 weeks; effective in approximately 65-70% of severe cases 1, 4
• Alternative agents for refractory disease:
  • Mycophenolate mofetil: Effective for both skin and systemic symptoms 4
  • Colchicine: First-choice agent for mild recurrent disease, though response rates are limited (approximately 30%) 4, 5
  • Anti-interleukin-1 agents: Consider for severe refractory cases 3
  • Cyclophosphamide, plasmapheresis, or intravenous immunoglobulin: Reserve for severe systemic disease unresponsive to other therapies 4, 5

Critical Treatment Pitfalls

• Avoid long-term oral corticosteroids as monotherapy due to cumulative toxicity including hypertension, hyperglycemia, osteoporosis, and gastric complications; always add steroid-sparing agents early 1, 4
• H1-antihistamines alone are ineffective in most UV patients (unlike ordinary chronic urticaria), with response rates not exceeding placebo in most studies; do not delay escalation to immunomodulatory therapy 4, 3
• Montelukast, H2-antihistamines, pentoxifylline, doxepin, and tranexamic acid are not effective in most UV patients and should not be relied upon 4
• Recognize that UV associated with drugs, malignancy, autoimmunity, or infections usually resolves with withdrawal or cure of the underlying condition; always investigate and address these causes 4

General Supportive Measures

• Minimize nonspecific aggravating factors including overheating, stress, and alcohol 1
• Avoid aspirin, NSAIDs, and codeine which can worsen symptoms 1
• Apply cooling antipruritic lotions (calamine or 1% menthol in aqueous cream) for symptomatic relief 1
• Provide patient education about the chronic nature and potential for eventual recovery 1

Monitoring Requirements

• Assess disease activity using the 7-Day Urticaria Vasculitis Activity Score (UVAS7) at every visit 3
• Monitor complement levels regularly in hypocomplementemic UV 1
• For patients on cyclosporine, monitor blood pressure and renal function every 6 weeks 1
• Consider stepping down therapy after achieving complete control for at least 3 consecutive months 1

Multidisciplinary Approach for Severe Cases

• Patients with hypocomplementemic UV or UVAS7 >7 require multidisciplinary management, particularly when underlying systemic lupus erythematosus, malignancy, or infection is present 3
• Coordinate care with rheumatology for systemic involvement and autoimmune disease evaluation 3