Department of Dermatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Department of Rheumatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
J Am Acad Dermatol. 2021 Dec;85(6):1503-1509. doi: 10.1016/j.jaad.2021.02.005. Epub 2021 Feb 5.
Small case series and case reports indicated that atypical persistent pruritic eruptions (PPEs), another type of skin lesions seen in adult-onset Still's disease (AOSD), imply a worse prognosis than typical evanescent rashes.
To investigate clinical characteristics and macrophage activation syndrome (MAS) occurrence in AOSD with PPEs.
A retrospective cohort study analyzed 150 patients with AOSD with rashes at the First Affiliated Hospital of Zhejiang University from January 2013 to December 2019.
Patients with AOSD with PPEs had higher lactate dehydrogenase (492.00 U/L vs 382.00 U/L; P < .001) and ferritin (6944.10 ng/ml vs 4286.60 ng/ml; P = .033), and lower fibrinogen (5.05 g/L vs 5.77 g/L; P = .014) than those with evanescent rashes. Patients with AOSD with PPEs had a higher incidence (17.4% vs 3.1%, P = .006) and cumulative event rate for MAS (P = .008) and tended to receive high-dose glucocorticoid (36% vs 20.3%; P = .036). Multivariate analysis indicated that PPEs (hazard ratio [HR], 5.519; 95% confidence interval [CI], 1.138-26.767; P = .034), aspartate aminotransferase of greater than 120 U/L (HR, 8.084; 95% CI, 1.728-37.826; P = .008), and splenomegaly (HR, 21.152; 95% CI, 2.263-197.711; P = .007) were independent risk factors for MAS.
Single-center, retrospective nature, small sample size.
PPEs indicated increased severity and MAS occurrence versus evanescent rashes. PPEs, aspartate aminotransferase of greater than 120 U/L, and splenomegaly were risk factors for MAS in AOSD with skin involvement.
小病例系列和病例报告表明，特发性持续性瘙痒性皮疹（PPEs）是成人Still 病（AOSD）中另一种皮肤损伤类型，其预后比典型一过性皮疹更差。
研究伴有 PPEs 的 AOSD 的临床特征和巨噬细胞活化综合征（MAS）发生情况。
回顾性队列研究分析了 2013 年 1 月至 2019 年 12 月在浙江大学第一附属医院就诊的 150 例伴有皮疹的 AOSD 患者。
与一过性皮疹患者相比，伴有 PPEs 的 AOSD 患者乳酸脱氢酶（492.00 U/L 比 382.00 U/L；P<0.001）和铁蛋白（6944.10 ng/ml 比 4286.60 ng/ml；P=0.033）更高，纤维蛋白原（5.05 g/L 比 5.77 g/L；P=0.014）更低。伴有 PPEs 的 AOSD 患者 MAS 的发生率（17.4%比 3.1%，P=0.006）和累积事件率更高（P=0.008），且更倾向于接受大剂量糖皮质激素治疗（36%比 20.3%；P=0.036）。多变量分析表明，PPEs（风险比[HR]，5.519；95%置信区间[CI]，1.138-26.767；P=0.034）、天门冬氨酸转氨酶大于 120 U/L（HR，8.084；95%CI，1.728-37.826；P=0.008）和脾肿大（HR，21.152；95%CI，2.263-197.711；P=0.007）是 MAS 的独立危险因素。
单中心、回顾性、样本量小。
与一过性皮疹相比，PPEs 提示疾病更严重且更易发生 MAS。伴有 PPEs、天门冬氨酸转氨酶大于 120 U/L 和脾肿大是伴有皮肤受累的 AOSD 发生 MAS 的危险因素。
