Hypersensitivity Vasculitis Treatment & Management

Updated: Apr 03, 2020
  • Author: Ruth Ann Vleugels, MD, MPH; Chief Editor: William D James, MD  more...
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Treatment

Medical Care

Once a diagnosis of hypersensitivity vasculitis (leukocytoclastic vasculitis) is established and the patient is fully evaluated, specific or nonspecific management options may be used.

Mild cases of hypersensitivity vasculitis are usually self-limited and treated with supportive care. Elevation of the legs or use of compression stockings may be helpful because the disease often affects dependent areas. NSAIDs, analgesics, or antihistamines can be used to treat symptoms of burning, pain, and pruritus.

Treat the cause in patients with an identifiable cause. Removal of a causative drug may result in rapid clearing of the process in as little as 2 weeks. In cases in which vasculitis is due to ongoing infection, avoid immunosuppressants and treat the underlying infection.

Treat chronic disease that primarily involves the skin with nontoxic modalities whenever possible; avoid using systemic corticosteroids and/or immunosuppressive agents. Colchicine [27, 28] or dapsone may be administered for patients with disease of the skin with or without joint manifestations.

Patients with urticarial lesions may be treated with antihistamines (both sedating and less sedating agents). Sometimes, a combination of these agents is needed to control disease manifestations. Some patients have also responded to nonsteroidal anti-inflammatory agents. However, a trial of colchicine, dapsone, or hydroxychloroquine may be required for disease control. [29]

Patients with bullous or ulcerating disease often require a short course of systemic corticosteroids to more rapidly achieve disease control. 

Patients with progressive or chronic cutaneous disease may require systemic corticosteroids and/or steroid-sparing agents such as azathioprine, methotrexate, [30, 31] or mycophenolate mofetil. Intravenous immunoglobulin has also been shown to be beneficial in isolated cases of chronic or refractory disease. [32]

Patients with severe visceral involvement may require high doses of systemic corticosteroids (1-2 mg/kg/day) with or without a steroid-sparing immunosuppressive agent (eg, cyclophosphamide, azathioprine, methotrexate, mycophenolate mofetil).

Rituximab use has been reported in various subsets of vasculitis patients, particularly those with antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis. Several patients with chronic cutaneous small-vessel vasculitis have also been treated effectively with this agent. [33, 34, 35]

Patients without other identifiable causes for hypersensitivity vasculitis can consider a restrictive/elimination diet, which can rarely identify a causative agent. [36]

In hypersensitivity vasculitis (leukocytoclastic vasculitis), inpatient care is needed for patients who have severe vasculitic syndromes with organ dysfunction. Most patients with cutaneous vasculitis are treated in an outpatient setting.

Consider transfer to a tertiary care facility for specialty care in patients with severe visceral disease or chronic unremitting cutaneous disease.

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Surgical Care

Surgical care is rarely needed for patients with hypersensitivity vasculitis (leukocytoclastic vasculitis). Surgical care may be appropriate if a tumor is identified as a cause of the process. Surgical care also may be appropriate if recalcitrant ulceration occurs after control of active disease.

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Consultations

The following consultations may be necessary:

  • Rheumatologist

  • Dermatologist

  • Nephrologist

  • Gastroenterologist/hepatologist

  • Pulmonologist

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Diet

No specific diet is required. A restrictive/elimination diet may be used for up to 2 weeks for diagnostic and therapeutic purposes. [36]

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Activity

No specific restrictions on activity are recommended. Leg elevation, however, may be helpful in patients with hypersensitivity vasculitis.

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Long-Term Monitoring

Design of a follow-up program depends on the vasculitic syndrome, its chronicity, and the organ systems affected. For some patients with hypersensitivity vasculitis, further follow-up care may not be needed once the process is inactive.

Patients with Henoch-Schönlein purpura may develop impairment of renal function or hypertension even after complete clearance of their skin disease; therefore, regular follow-up care is needed. [37]

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