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.
Finkelstein-Seidlmayer vasculitis, also referred to as acute hemorrhagic edema of young children, is a rare small-vessel leukocytoclastic vasculitis. This condition is skin-limited, mainly affects infants up to 2 years of age and spontaneously remits. It has been suggested that an infection or a vaccine precede (by ≤ 14 days) this vasculitis. To better understand the interplay between infections or vaccines and Finkelstein-Seidlmayer vasculitis, we utilized the data contained in the Acute Hemorrhagic Edema BIbliographic Database AHEBID. The database, initiated in 2019, is being regularly updated, encompasses the entire original literature on Finkelstein-Seidlmayer vasculitis published after the original description and is attainable on request. The possible existence of an infectious or a vaccine precursor was addressed in 447 cases. Most cases were preceded by an infection (N = 384; 86%), by a vaccination (N = 20; 4.4%), or both an infection and a vaccination (N = 17; 3.8%). No precursor was reported in the remaining cases (N = 26; 5.8%). Two distinct infections preceded the onset of the vasculitis in 11 of the 381 cases with infection-associated Finkelstein-Seidlmayer vasculitis. The following infectious precursors were reported: upper respiratory tract infection (N = 292); acute gastroenteritis (N = 40); a benign febrile infection (N = 36); lower respiratory tract infection (N = 22); further infections (N = 8). The temporal relationship between the infectious precursor and the onset of the skin eruption was detailed in 336 cases: 54 cases developed before resolution and 282 after resolution of the infection. In conclusion, most cases of Finkelstein-Seidlmayer vasculitis are preceded by an infection. In a minority of cases, this skin vasculitis develops before resolution of the infection. In most cases, however, this vasculitis develops after resolution of the infection. More rarely, this vasculitis is preceded by a vaccination.
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