Challenging Clinical Therapeutic Approach to Urticarial Vasculitis: A Case Series

Background: Urticarial vasculitis (UV) is a rare form of small-vessel vasculitis characterized by persistent urticarial lesions and systemic manifestations. It differs from chronic spontaneous urticaria (CSU) both clinically and pathogenetically, often requiring systemic therapy. Despite its complexity, no standardized treatment algorithm exists. Methods: We conducted a retrospective, monocentric, observational study on 11 patients diagnosed with UV at the Dermatology Unit of Tor Vergata University Hospital (Rome) between 2014 and 2024. Demographic, clinical, serological, and therapeutic data were collected from medical records. The therapeutic response was assessed using the Urticaria Control Test (UCT) score. Results: The cohort comprised predominantly women (91%), with a mean age at diagnosis of 52.8 years. Autoimmune thyroiditis was the most frequent comorbidity (64%). Hypocomplementemia was detected in only one patient (9%), who also had systemic lupus erythematosus. Antihistamines, while usually prescribed, showed limited efficacy since none of the patients achieved complete remission with monotherapy. Systemic corticosteroids demonstrated rapid and effective control in acute phases. Omalizumab produced variable responses, with two patients achieving a high response (HR) and one reaching complete remission (CR). Methotrexate and cyclosporine yielded inconsistent outcomes. Due to the heterogeneity and limited sample size, statistical analyses were not performed. Conclusions: UV presents with diverse clinical profiles and therapeutic responses. No treatment proved universally efficacious, but corticosteroids and omalizumab were effective in an acute and maintenance phase, respectively. Our findings underscore the importance of individualized treatment plans and the need for further studies to define predictive biomarkers and therapeutic strategies in UV.