报道1例8岁6月龄女性患儿，以反复全身性荨麻疹样皮损及弛张高热为主要表现，初期拟诊为“慢性荨麻疹”，经常规抗感染、抗组胺及糖皮质激素治疗效果不佳，病程中出现一过性关节痛、多浆膜腔积液、淋巴结肿大表现，并激素减量后迅速出现高炎症状态及血小板降低、铁蛋白显著升高、高甘油三酯及低纤维蛋白原等改变，在排除感染、肿瘤性疾病后，最终诊断为全身型幼年特发性关节炎（SJIA）合并巨噬细胞活化综合征（MAS）。患儿经大剂量激素冲击、环孢素及芦可替尼等多靶点免疫抑制治疗后病情逐渐控制。本文结合该病例，重点探讨以荨麻疹样皮损为首发表现疾病的鉴别诊断、诊断思路及早期识别策略，旨在提升儿科医生提高对此类表现疾病的诊疗能力。

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.