Skin Eruptions in Acute Hemorrhagic Edema of Young Children: Systematic Review of the Literature

Abstract

<b><i>Background:</i></b> Acute hemorrhagic edema is a skin-limited small-vessel leukocytoclastic vasculitis, which affects infants 4 weeks to 2 years of age and remits within 3 weeks. The diagnosis is made clinically in not-ill appearing children with acute onset of raised annular or nummular eruptions and edema. In this vasculitis, type, distribution, and evolution of the rash have never been systemically investigated. To address this issue, we employed the data contained in the Acute Hemorrhagic Edema Bibliographic Database, which incorporates all reports on acute hemorrhagic edema. <b><i>Summary:</i></b> Key features of rash were documented in 383 children. Annular eruptions in a strict sense, usually targetoid, were reported in 375 (98%) cases (many children also presented polycyclic or arciform eruptions). Nummular eruptions were also very common (<i>n</i> = 358; 93%). Purpuric eruptions and ecchymoses were reported in the vast majority of cases. Macules and wheals were described in a minority of cases. Edema, detected in all cases, was mostly painful, indurated and nonpitting. The following regions were affected, in decreasing order, by annular or nummular eruptions: legs, feet, face, arms, ears, trunk, and genitals. With the exception of feet, which were very often affected, the same distribution was reported for edema. The initial eruption was often a wheal or a macule that evolved into a nummular or an annular eruption. Nummular eruptions successively evolved into annular ones. <b><i>Key Message:</i></b> This study carefully characterizes type, distribution, and evolution of skin eruption in acute hemorrhagic edema. The data help physicians to rapidly and noninvasively make the clinical diagnosis of this vasculitis.

Köbner and Pastia Signs in Acute Hemorrhagic Edema of Young Children: Systematic Literature Review

“…The Köbner sign is a well-recognized clinical sign that is mainly caused by mechanical friction [ 4 ]. The localization we noted in this review and the patients’ age suggest that in AHE, this sign is mainly brought about by diapers and socks.…”

“…Our findings are in line with the literature. The Köbner sign occurs in approximately 10% of patients with a skin-limited or skin-predominant vasculitis [ 4 ]. Furthermore, this sign has been recently identified in approximately every fourth child with Henoch–Schönlein purpura, the most common childhood vasculitis [ 90 ].…”

“…AHEBID was created in 2019 and incorporates the original articles on AHE published after the original report in 1913 [ 3 , 4 , 10 ]. The database is obtainable on request (email: finkelstein-seidlmayer@usi.ch ).…”

“…Acute hemorrhagic edema of young children (AHE), often referred to as acute cockade purpura and edema, Finkelstein–Seidlmayer syndrome or infantile Henoch–Schönlein syndrome, is a skin-limited small-vessel leukocytoclastic vasculitis [ 1 , 2 , 3 , 4 ] that typically occurs after a simple, mostly viral, febrile illness (more rarely after a vaccine). It predominantly affects children 4 weeks to 2 years of age, spontaneously remits within 3 weeks and does not recur [ 1 , 2 , 3 , 4 ]. Recent data point out that a mild and self-remitting abdominal, articular or renal involvement occurs in about 15% of cases [ 3 ].…”

“…The data contained in the A cute H emorrhagic E dema BI bliographic D atabase AHEBID [ 3 , 4 , 10 ] were employed to investigate the prevalence, characteristics, and clinical relevance of the Köbner and Pastia signs in AHE.…”