Chronic spontaneous urticaria is commonly responsive to high-dose antihistamines, anti-IgE therapy, and cyclosporine; however, a subset of patients have persistent symptoms despite guideline-directed treatment. We present a case of long-standing, treatment-refractory urticaria in a 38-year-old man with persistent neutrophilia and elevated inflammatory markers. The clinical presentation prompted consideration of chronic spontaneous urticaria mimics, including urticarial vasculitis, autoimmune disease, hematologic disease, and autoinflammatory syndromes. Further evaluation included skin biopsy, expanded laboratory testing, and additional targeted investigations. This case highlights the importance of broadening the differential diagnosis in patients with chronic urticaria that is refractory to standard therapy, particularly when systemic inflammatory features are present.
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Sivaloganathan et al. (2026) studied this question.
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