What is the management approach for leukocytoclastic vasculitis?

From the Guidelines

The management of leukocytoclastic vasculitis (LCV) should focus on identifying and treating the underlying cause, controlling inflammation, and preventing organ damage, with corticosteroids being the mainstay of therapy for symptomatic cases, as supported by the general principles of vasculitis management 1. The treatment approach for LCV involves several key steps, including:

• Removing any triggering factors such as medications, treating underlying infections, or managing associated conditions
• Providing supportive care with leg elevation, compression stockings, and antihistamines for mild cutaneous disease limited to the skin
• Using corticosteroids, such as prednisone, at a dose of 0.5-1 mg/kg/day for 1-2 weeks, followed by a gradual taper over 4-6 weeks, for more symptomatic cases
• Considering steroid-sparing agents, including colchicine, dapsone, or hydroxychloroquine, for recurrent or refractory cases
• Using immunosuppressive agents, such as azathioprine, methotrexate, mycophenolate mofetil, or cyclophosphamide, for severe cases with systemic involvement or organ damage, as recommended for ANCA-associated vasculitis 1
• Regular monitoring for medication side effects and disease activity is essential, and treatment duration depends on clinical response and the underlying cause It is also important to note that patients with LCV may develop complications from treatment many years after discontinuing treatment, and long-term follow-up and rapid access to specialist services are necessary for all patients with vasculitis, as emphasized in the management of ANCA-associated vasculitis 1.

From the Research

Management Approach for Leukocytoclastic Vasculitis

The management approach for leukocytoclastic vasculitis (LCV) depends on the underlying cause and the extent of systemic involvement. The following are key points to consider:

• Symptom Management: The treatment is mainly focused on symptom management, based on rest, low dose corticosteroids, colchicine or different unproven therapies, if skin-limited 2.
• Discontinuation of Culprit Drug: When a medication is the cause, the prognosis is favorable and the discontinuation of the culprit drug is usually resolutive 2.
• Systemic Vasculitis: When a systemic vasculitis is the cause of LCV, higher doses of corticosteroids or immunosuppressive agents are required, according to the severity of organ involvement and the underlying associated disease 2.
• Chronic or Relapsing LCV: Colchicine or dapsone may be used as first-line or second-line therapy for chronic or relapsing LCV 3, 4.
• Severe Systemic Vasculitis: Severe systemic vasculitis requires immunosuppressive strategies, such as glucocorticoids and cyclophosphamide 3, 4.

Diagnostic Guidelines

The aim of diagnostic guidelines is to determine the specific type and systemic involvement of LCV and to identify an underlying cause. The following are key points to consider:

• Basic Work-up: Basic work-up should encompass history of drug intake and of preceding infections, biopsy with immunofluorescence, differential blood count, urine analysis and throat swabs 3.
• Histopathological Examination: The diagnosis relies on histopathological examination, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments ("leukocytoclasia") 2.
• Serological Tests: Serological tests for hepatitis B and C viruses, autoantibodies (anti-nuclear antibodies and anti-neutrophil cytoplasmic antibodies), complement fractions and IgA staining in biopsy specimens are part of the usual work-up of LCV 2.

Treatment Options

The treatment options for LCV depend on the underlying cause and the extent of systemic involvement. The following are key points to consider:

• Corticosteroids: Corticosteroids are indicated when there are signs of incipient skin necrosis or ulceration 3, 4, 5.
• Colchicine or Dapsone: Colchicine or dapsone may be used as first-line or second-line therapy for chronic or relapsing LCV 3, 4.
• Immunosuppressive Agents: Immunosuppressive agents, such as glucocorticoids and cyclophosphamide, are required for severe systemic vasculitis 3, 4.