Reviews and feature article
Treatment of urticarial vasculitis: A systematic review

https://doi.org/10.1016/j.jaci.2018.09.007Get rights and content

Urticarial vasculitis (UV) is a difficult-to-treat condition characterized by long-lasting urticarial rashes and histopathologic findings of leukocytoclastic vasculitis. Treatment is dictated by the severity of skin and systemic involvement and the underlying systemic disease. This is a comprehensive systematic review of the efficacy of current UV treatment options. We searched for relevant studies in 7 databases, including MEDLINE, Scopus, and Web of Science. In total, 261 eligible studies and 789 unique patients with UV were included in the systematic review. Most patients with UV are adult women with chronic (≥6 weeks) and systemic disease. UV is mostly idiopathic but can be associated with drugs, malignancy, autoimmunity, and infections. It usually resolves with their withdrawal or cure. Corticosteroids are effective for the treatment of skin symptoms in more than 80% of patients with UV. However, their long-term administration can lead to potentially serious adverse effects. The addition of immunomodulatory or immunosuppressive agents often allows corticosteroid tapering and improves the efficacy of therapy. Biologicals, including omalizumab, as well as corticosteroids, cyclophosphamide, dapsone, mycophenolate mofetil, plasmapheresis, colchicine, hydroxychloroquine, intravenous immunoglobulin, nonsteroidal anti-inflammatory drugs, and cyclosporine, can be effective for both skin and systemic symptoms in patients with UV. H1-antihistamines, montelukast, danazol, H2-antihistamines, pentoxifylline, doxepin, and tranexamic acid are not effective in most patients with UV. As of yet, no drugs have been approved for UV, and management recommendations are based mostly on case reports and retrospective studies. Prospective studies investigating the effects of treatment on the signs and symptoms of UV are needed.

Section snippets

Search strategy

The systematic literature review was performed according to PRISMA guidelines.32 A literature search of MEDLINE, Scopus, Web of Science, Biosis Citation Index, KCI Korean Journal Database, Russian Science Citation Index, and SciELO Citation Index databases (from January 1940 to December 2017) was conducted by using the following terms: “urticarial vasculitis” OR “hypocomplementemic vasculitis” OR “normocomplementemic vasculitis” OR “hypocomplementic vasculitis” OR “anti-C1q vasculitis” OR

Most patients with UV are adult women with chronic and systemic disease

In total, 261 eligible studies and 789 patients with UV were included in the systematic review (Fig 1 and see Tables E3 and E5 in this article's Online Repository at www.jacionline.org). Most studies came from the United States (n = 81), the United Kingdom (n = 32), France (n = 20), Japan (n = 19), Italy (n = 16), and Germany (n = 15). Forty-one studies reported UV in more than 1 patient (7 prospective studies33, 34, 35, 36, 37, 38, 39 and 34 retrospective studies). Most patients were adult

Does the treatment of underlying disease lead to UV remission?

Our findings indicate that UV can be caused by drugs, malignancy, and infections and that their withdrawal and cure, respectively, can lead to UV resolution. For example, Kulthanan et al12 reported that drugs, most commonly nonsteroidal anti-inflammatory drugs, elicit UV in 7.8% of patients with UV. Drug reactions usually occurred in adult patients with acute NUV within 3 to 10 days after administration of the drug, and remission of UV was seen within a month after the drug was discontinued.7,

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    P.K. received European Academy of Dermatology and Venereology Research Fellowship (RF-2017-24) and was supported by the “Russian Academic Excellence Project 5-100.”

    Disclosure of potential conflict of interest: The authors declare that they have no relevant conflicts of interest.

    ∗

    These authors contributed equally to this work and should be considered co-first authors.

    ‡

    These authors contributed equally to this work and should be considered co-senior authors.

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