Abstract:

This article reports the case of an 8-year-and-6-month-old female who presented with recurrent generalized urticarial lesions and remittent high fever. Initially suspected as chronic urticaria, her condition showed poor response to conventional treatments including anti-infectives, antihistamines, and glucocorticoids. The disease progressed rapidly with multisystem involvement and a marked elevation in inflammatory markers. Through multidisciplinary collaboration and systematic evaluation, she was ultimately diagnosed with systemic juvenile idiopathic arthritis (SJIA) complicated by macrophage activation syndrome (MAS). Her condition was gradually controlled with multitarget immunosuppressive therapy, including high-dose glucocorticoid pulse therapy, cyclosporine, and ruxolitinib. Based on this case, this article focuses on the differential diagnosis, diagnostic approach, and early recognition strategies for diseases presenting initially with urticarial lesions, aiming to enhance pediatricians' ability in the diagnosis and management of such conditions, thereby enabling early screening, intervention, and improved prognosis for complex and critical cases.

Key words: urticaria, systemic juvenile idiopathic arthritis, macrophage activation syndrome