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.
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 
