Medical Care
Once a diagnosis of leukocytoclastic vasculitis (LCV) is established and the patient is fully evaluated, specific or nonspecific management options may be used, depending on the etiology and the extent of the disease. Possible interventions include the following [43, 44] :
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LCV often affects dependent areas; thus, elevation of the legs or compression stockings may be useful
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Patients with an identifiable cause should receive treatment for that cause. [45] Removal of a drug thought to be causing LCV may result in rapid clearing of the process in as little as 2 weeks
Patients with urticarial lesions may be treated with antihistamines, including both sedating and less-sedating agents. However, these patients may fail to respond to antihistamines, and often a trial of colchicine or dapsone may be needed. In some cases, a combination of these agents is needed to control the disease manifestations. Some patients respond to nonsteroidal anti-inflammatory agents.
Patients with severe visceral involvement may require high doses of corticosteroids (1-2 mg/kg/d) with or without an immunosuppressive agent (eg, cyclophosphamide, azathioprine, methotrexate, mycophenolate mofetil, rituximab). Patients with bullous lesions may require a brief course of systemic corticosteroids in order to gain rapid control and minimize ulcer formation at the sites of bullae. Due to the possible adverse effects of prolonged corticosteroid use, other treatments should be considered if long-term treatment is needed. [49]
Treatment of patients with chronic LCV is a challenge. Patients may attempt a restrictive elimination diet, which rarely can allow for identification of the cause and control of the disease. [50]
Colchicine at 0.6 mg twice daily and/or dapsone at 100-200 mg/d may control the disease. The combination of those two agents seems to be complementary. In patients whose conditions do not respond or respond poorly, other agents may be administered, including immunosuppressive or cytotoxic agents, (eg methotrexate, mycophenolate mofetil, azathioprine). Patients with severe or debilitating disease might also be treated with biologic agents such as rituximab or intravenous immunoglobulin. Of note, these agents are rarely needed for cutaneous LCV.
In a multicenter, randomized, double-blind trial, Stone et al found that rituximab (375 mg/m2/wk for 4 wk) was more efficacious than cyclophosphamide (2 mg/kg/d) for inducing remission of relapsing ANCA-associated vasculitis. Prednisone was gradually tapered downward; 67% of the rituximab group compared with 42% of the cyclophosphamide group reached the primary end point, which was remission of disease without use of prednisone at 6 months (P = 0.01). [51]
Inpatient care is needed in patients who have severe systemic vasculitic syndromes and severe organ dysfunction. Most patients with cutaneous LCV are treated in an outpatient setting.
Surgical Care
Surgical care is rarely needed in patients with vasculitis. However, surgery may be appropriate in any of the following circumstances:
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A tumor is identified as a cause of the process
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A recalcitrant ulceration persists after the active disease has been controlled
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An organ biopsy is needed
Consultations
The following specialty consultations may be indicated:
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Rheumatologist
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Dermatologist
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Nephrologist
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Gastroenterologist or hepatologist
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Immunologist or allergist
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Pulmonologist
Diet and Activity
No specific diet is required in patients with leukocytoclastic vasculitis. A restrictive elimination diet may be considered for diagnostic and therapeutic purposes. [50]
No specific restrictions on activity are necessary in patients with leukocytoclastic vasculitis. However, leg elevation may be helpful.
Long-Term Monitoring
Transfer to a tertiary care facility should be considered in patients with severe visceral disease. Patients with chronic LCV are often referred to a tertiary care center for specialty care.
The design of a follow-up program depends on the vasculitic syndrome, its chronicity, and the organ systems affected. Once the process is inactive in a patient with leukocytoclastic vasculitis (LCV), further follow-up care may be unnecessary.
Patients with Henoch-Schönlein purpura may develop impaired renal function or hypertension; therefore, regular follow-up care, even after complete clearing of their disease, is needed.
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Hypersensitivity vasculitis.
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Henoch-Schönlein purpura.
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Histopathology of leukocytoclastic vasculitis.
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Urticarial vasculitis. Lesions differ from routine urticaria (hives) in that they last longer (often >24 h), are less pruritic, and often resolve with a bruise or residual pigmentation.
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Erythema elevatum diutinum, a rare cutaneous vasculitis.
