DOI: https://doi.org/10.36347/sjmcr.2026.v14i04.022

Pages: 678-679

Background: Urticaria is a common inflammatory mucocutaneous syndrome with multiple etiologies. When recalcitrant to standard antihistamine therapy, a systematic diagnostic workup is essential to identify rare underlying systemic conditions such as Schnitzler Syndrome. Case Presentation: We report the case of a 41-year-old woman with a background of chronic asthma, fibromyalgia, degenerative retinopathy, and nasal polyposis, who presented with an 8-month history of recalcitrant urticarial eruption. Despite sequential trials of multiple antihistamine regimens including cetirizine, hydroxyzine, loratadine, levocetirizine, desloratadine, montelukast, and bilastine at standard and quadruple doses no clinical improvement was observed. Extensive etiological workup excluded common chronic urticaria, drug-induced, infectious, inducible, and autoimmune causes, as well as urticarial vasculitis, Adult-onset Still's disease, systemic lupus erythematosus, and Cryopyrin-Associated Periodic Syndromes (CAPS). Serum protein electrophoresis revealed a monoclonal peak in the gamma-globulin zone, confirmed by immunofixation as monoclonal IgG elevation, establishing the diagnosis of Schnitzler Syndrome. Treatment and Outcome: The patient was initiated on corticosteroid therapy with prednisolone 40 mg/day for 3 months, resulting in marked clinical improvement. Conclusion: This case highlights the importance of a thorough diagnostic approach in chronic recalcitrant urticaria. While antihistamines remain the first-line treatment, corticosteroid therapy may play a critical role in antihistamine-refractory forms, particularly in the context of Schnitzler Syndrome. Clinicians should consider monoclonal gammopathy screening in patients with persistent urticaria unresponsive to standard therapy.