Medication Summary
No standard therapeutic protocol exists for hypersensitivity vasculitis. In addition, few of the therapies currently used for this entity have been tested in controlled trials.
Anti-inflammatories
Class Summary
These agents decrease inflammatory responses and systemically interfere with events leading to inflammation.
Colchicine
Colchicine has effects against neutrophils, which are involved in the pathogenesis of hypersensitivity vasculitis. Colchicine has been demonstrated to be steroid-sparing in open-label studies. The only double-blinded placebo-controlled trial failed to demonstrate its efficacy; however, several methodological errors occurred in this study. It is not FDA approved in children.
Dapsone (Avlosulfon)
Small open-label studies or single case reports have suggested that dapsone is effective in some patients with cutaneous vasculitis. Dapsone is used in hypersensitivity vasculitis not for its antimicrobial activity but for its modulatory effect on neutrophil activity.
Corticosteroids
Class Summary
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.
Prednisone (Deltasone)
Prednisone is indicated for vasculitis affecting internal organs (eg, kidneys, lungs, CNS). Patients with bullous skin lesions require corticosteroid therapy in order to prevent cutaneous ulceration.
Cytotoxic/immunosuppressives
Class Summary
These agents inhibit cell growth and proliferation. Agents in this class possibly used in hypersensitivity vasculitis include cyclophosphamide, azathioprine, methotrexate, rituximab, and mycophenolate.
Cyclophosphamide (Cytoxan, Neosar)
Cyclophosphamide is useful in life-threatening cases of vasculitis. Patients with skin-limited disease generally should not be treated with this agent. It is useful in patients with granulomatosis with polyangiitis (formerly Wegener granulomatosis), polyarteritis nodosa, or eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). Cyclophosphamide is an alkylating agent that depresses T- and B-cell function.
Azathioprine (Imuran)
Azathioprine antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. It may decrease the proliferation of immune cells, which results in lower autoimmune activity.
Methotrexate (Folex, Rheumatrex)
Methotrexate inhibits 5-aminoimidazole-4-carboxamide ribonucleotide (AICAR) tranformylase, which leads to increased levels of adenosine. This increase in adenosine inhibits leukocyte accumulation, thereby decreasing inflammation. Methotrexate ameliorates symptoms of inflammation (eg, pain, swelling, stiffness). Adjust the dose gradually to attain a satisfactory response.
Rituximab (Rituxan)
Rituximab is a genetically engineered human monoclonal antibody directed against the CD20 antigen found on the surface of normal and malignant B lymphocytes. It immunomodulates response against malignant cells.
Mycophenolate (CellCept)
Mycophenolate inhibits inosine monophosphate dehydrogenase (IMPDH) and suppresses de novo purine synthesis by lymphocytes, thereby inhibiting their proliferation. Inhibits antibody production.
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Hypersensitivity vasculitis.
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Henoch-Schönlein purpura.
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Histopathologic features of leukocytoclastic vasculitis.
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Urticarial vasculitis. These lesions differ from routine hives by lasting longer (often >24 h), being less pruritic, and often resolving with a bruise or residual pigmentation.
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Erythema elevatum diutinum, a rare cutaneous vasculitis.
