Bronz Gabriel, Consolascio Danilo, Bianchetti Mario G, Rinoldi Pietro O, Betti Céline, Lava Sebastiano A G, Milani Gregorio P
Pediatric Institute of Southern Switzerland, Ospedale San Giovanni, Ente Ospedaliero Cantonale, 6500 Bellinzona, Switzerland.
Family Medicine Institute, Faculty of Biomedical Sciences, Università della Svizzera Italiana, 6900 Lugano, Switzerland.
Children (Basel). 2022 Feb 15;9(2):265. doi: 10.3390/children9020265.
Acute hemorrhagic edema of young children, a benign skin-limited vasculitis, predominantly affects children 2 years of age or younger. The prevalence and clinical features of the Köbner and Pastia signs have never been systematically investigated in this vasculitis. To address this issue, we analyzed the data contained in the Acute Hemorrhagic Edema Bibliographic Database, which incorporates all reports on hemorrhagic edema published after 1969. A total of 339 cases (236 males and 103 females; 11 (8-18) months of age; median and interquartile range) were documented with at least 1 photograph and therefore included in this analysis. The Köbner sign occurred in 24 cases (14 males and 10 females; 11 (7-17) months of age), the Pastia sign in 51 cases (39 males and 12 females; 11 (8-15) months of age), and both Köbner and Pastia signs in 8 cases (7 males and 1 female; 11 (7-17) months of age). The lower legs, thighs, waistline, and groin were the regions that were most commonly affected with the Köbner sign, while the ankle, feet, cubital fossa, and elbow were most affected with the Pastia sign. The Köbner and Pastia signs are clinically relevant; they occur in about every fourth child affected with hemorrhagic edema and do not influence the disease progression.
小儿急性出血性水肿是一种局限于皮肤的良性血管炎，主要影响2岁及以下儿童。在这种血管炎中，同形反应（Köbner征）和帕氏线（Pastia征）的患病率及临床特征从未得到系统研究。为解决这一问题，我们分析了急性出血性水肿文献数据库中的数据，该数据库纳入了1969年后发表的所有关于出血性水肿的报告。共有339例病例（236例男性和103例女性；年龄中位数为11（8 - 18）个月；四分位间距）有至少1张照片记录，因此纳入本分析。同形反应（Köbner征）出现在24例（14例男性和10例女性；年龄11（7 - 17）个月），帕氏线（Pastia征）出现在51例（39例男性和12例女性；年龄11（8 - 15）个月），同形反应（Köbner征）和帕氏线（Pastia征）均出现的有8例（7例男性和1例女性；年龄11（7 - 17）个月）。小腿、大腿、腰围和腹股沟是同形反应（Köbner征）最常累及的部位，而踝关节、足部、肘窝和肘部是帕氏线（Pastia征）最常累及的部位。同形反应（Köbner征）和帕氏线（Pastia征）具有临床相关性；约每四分之一患出血性水肿的儿童会出现这些体征，且不影响疾病进展。
