Skin eruptions in acute hemorrhagic edema of young children

Bronz, Gabriel; Rinoldi, Pietro O.; Lavagno, Camilla; Bianchetti, Mario; Lava, Sebastiano A. G.; Vanoni, Federica; Milani, Gregorio P.; Terrani, Isabella; Ferrarini, Alessandra

doi:10.1159/000519009

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Journal article

Skin eruptions in acute hemorrhagic edema of young children : systematic review of the literature

Bronz, Gabriel ORCID Pediatric Institute of Southern Switzerland, Ospedale San Giovanni, Bellinzona, Switzerland ; Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland
Rinoldi, Pietro O. Pediatric Institute of Southern Switzerland, Ospedale San Giovanni, Bellinzona, Switzerland
Lavagno, Camilla Pediatric Institute of Southern Switzerland, Ospedale San Giovanni, Bellinzona, Switzerland
Bianchetti, Mario ORCID Pediatric Institute of Southern Switzerland, Ospedale San Giovanni, Bellinzona, Switzerland ; Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland
Lava, Sebastiano A. G. Pediatric Cardiology Unit, Department of Pediatrics, Centre Hospitalier Universitaire Vaudois and University of Lausanne, Lausanne, Switzerland
Vanoni, Federica Pediatric Institute of Southern Switzerland, Ospedale San Giovanni, Bellinzona, Switzerland ; Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland
Milani, Gregorio P. Pediatric Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy ; Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milan, Italy
Terrani, Isabella Department of Dermatology, Ente Ospedaliero Cantonale, Ospedale Regionale di Lugano, Lugano, Switzerland
Ferrarini, Alessandra Service of Medical Genetics, Ospedale Italiano di Lugano, Lugano, Switzerland

2022

Published in:

Dermatology. - 2022, vol. 238, p. 397-403

Finkelstein-Seidlmayer disease

English Background: 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. Summary: 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 (n = 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. Key Message: This study carefully characterizes type, distribution, and evolution of skin eruption in acute hemorrhagic edema. The data help physicians to rapidly and non-invasively make the clinical diagnosis of this vasculitis.

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