Long-Term Efficacy and Safety of Omalizumab Monotherapy in a Patient With Normocomplementemic Urticarial Vasculitis

October 2022 | Volume 21 | Issue 10 | 1124 | Copyright © October 2022


Published online September 22, 2022

doi:10.36849/JDD.6739

Mohamed Abuzakouk MDa, Omar Ghorab MB Bch BAOb, Rajaie Namas MDc, Marcus Maurer MDd

aAllergy and Immunology Department, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
bSchool of Medicine, Royal College of Surgeons in Ireland, Bahrain, Kingdom of Bahrain
cRheumatology Department, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
dDermatological Allergology, Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Berlin, Germany

Abstract
Normocomplementemic urticarial vasculitis is a rare autoimmune disorder characterized by leukocytoclasia, fibrin deposits, and extravasated erythrocytes affecting multiple organ systems. Current treatment modalities, including corticosteroids and immunosuppressive agents, are of limited efficacy and an expansive side effect profile. Omalizumab has been reported to be effective in urticarial vasculitis, but its long-term effectiveness and tolerability have not yet been evaluated. We report a case of long-standing normocomplementemic urticarial vasculitis treated with omalizumab only, for almost 3 years. The patient reported a significant improvement in quality of life after the first few doses with a significant improvement in the urticaria control test. The treatment was well tolerated and no adverse events were reported after 3 years. Our patient was treated with 300 mg of omalizumab, as it was previously linked with a better improvement in quality of life. We were able to extend our patient’s treatment intervals, suggesting that this is feasible in patients treated with omalizumab who achieve a complete response. We recommend that larger and long-term studies are conducted to assess the efficacy and effectiveness of omalizumab in patients with urticarial vasculitis.

J Drugs Dermatol. 2022;21(10):1124-1126. doi:10.36849/JDD.6739

INTRODUCTION

Normocomplementemic urticarial vasculitis (NUV), a rare disease of long duration, presents with long-lasting wheals that show histopathologic features of vasculitis including leukocytoclasia, fibrin deposits, and extravasated erythrocytes.1,2,3 The musculoskeletal, respiratory, renal, and gastrointestinal systems can be involved.3 Most commonly, NUV is an idiopathic disease, but it may also occur in association with other autoimmune disorders such as systemic lupus erythematosus and Sjogren’s syndrome.3

To date, there is no approved treatment for NUV, and the management remains challenging in many patients.4 Different therapeutic agents are used in clinical practice including corticosteroids, cyclophosphamide, dapsone, mycophenolate mofetil, colchicine, hydroxychloroquine, and other immunosuppressants, often with limited benefit and considerable side effects.4 In a recent worldwide study, the limited efficacy of these medications was among the greatest perceived challenges reported by physicians who treat patients with NUV.5

Omalizumab, an anti-IgE mAb licensed for the treatment of chronic spontaneous urticaria (CSU) and asthma, has been reported to be effective in some patients with NUV.4,6,7,8 In a recent investigator-initiated open-label proof-of-concept study, 74% of 23 patients with NUV showed a beneficial response to omalizumab after 12 weeks of treatment.9 What is presently unclear is the long term efficacy and safety of omalizumab in the treatment of patients with NUV.

CASE

In May of 2018, a 33-year-old male presented to our urticaria center of reference and excellence (UCARE)10 at Cleveland Clinic Abu Dhabi, with an 8-year history of recurrent skin rashes affecting his forearms and lower legs (Figure 1). The lesions
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