Department of Dermatology, Singapore General Hospital, Singapore.
Dermatol Online J. 2023 Aug 15;29(4). doi: 10.5070/D329461904.
Neutrophilic urticarial dermatosis is a distinct entity strongly associated with underlying autoinflammatory disease. The pathogenesis of this condition has been considered to center around interleukin-1. We report a young woman with neutrophilic urticarial dermatosis who presented with a recurrent urticarial rash for two years prior to the onset of other systemic features including persistent fevers, sore throat, leukocytosis, elevated ferritin, and splenomegaly. She was ultimately diagnosed with adult-onset Still disease and responded well to treatment with systemic corticosteroids. Although neutrophilic urticarial dermatosis is known to occur in the setting of systemic symptoms and disease, its occurrence preceding the onset of systemic inflammation is less well-described in current literature.
中性粒细胞性荨麻疹性皮病是一种与潜在自身炎症性疾病密切相关的独特实体。该病症的发病机制被认为围绕白细胞介素-1展开。我们报告了一例中性粒细胞性荨麻疹性皮病年轻女性患者，她在出现其他全身症状（包括持续发热、咽痛、白细胞增多、铁蛋白升高和脾肿大）之前，已有两年反复发作的荨麻疹性皮疹病史。最终，她被诊断为成人Still 病，全身用皮质类固醇治疗效果良好。尽管已知中性粒细胞性荨麻疹性皮病可在全身症状和疾病的背景下发生，但在当前文献中，其在全身性炎症发作之前发生的情况描述较少。
