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Published in: European Journal of Pediatrics 4/2016

01-04-2016 | Original Article

Acute hemorrhagic edema of young children: a prospective case series

Authors: Alessandra Ferrarini, Cecilia Benetti, Pietro Camozzi, Alessandro Ostini, Giacomo D. Simonetti, Gregorio P. Milani, Mario G. Bianchetti, Sebastiano A. G. Lava

Published in: European Journal of Pediatrics | Issue 4/2016

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Abstract

Acute hemorrhagic edema of young children is a rare leukocytoclastic vasculitis that has been reported exclusively in small retrospective cases series, case reports, or quizzes. Considering that retrospective experience deserves confirmation in at least one observational prospective study, we present our experience with 16 children (12 boys and 4 girls, 5–28 months of age) affected by acute hemorrhagic edema. The patients were in good general conditions and with a low-grade or even absent fever. They presented with non-itching red to purpuric targetoid lesions not changing location within hours, with non-pitting and sometimes tender indurative swelling, and without mucous membrane involvement or scratch marks. Signs for articular, abdominal, or kidney involvement were absent. Antinuclear or antineutrophil cytoplasmic autoantibodies were never detected. The cases were managed symptomatically as outpatients and fully resolved within 4 weeks or less. No recurrence or familiarity was noted.
Conclusion: This is the first prospective evaluation of hemorrhagic edema. Our findings emphasize its distinctive tetrad: a well-appearing child; targetoid lesions that do not change location within hours; non-pitting, sometimes tender edema; complete resolution without recurrence.
What is known
Acute hemorrhagic edema of young children is considered a benign vasculitis.
There have been ≈100 cases reported in small retrospective case series.
What is new
The first prospective evaluation of this condition emphasizes its features: febrile prodrome; well-appearing child; targetoid lesions not changing location within hours; non-pitting, sometimes tender indurative edema; absent extracutaneous involvement; resolution within 3 weeks.
Antineutrophil cytoplasmic autoantibodies do not play a pathogenic role.
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Metadata
Title
Acute hemorrhagic edema of young children: a prospective case series
Authors
Alessandra Ferrarini
Cecilia Benetti
Pietro Camozzi
Alessandro Ostini
Giacomo D. Simonetti
Gregorio P. Milani
Mario G. Bianchetti
Sebastiano A. G. Lava
Publication date
01-04-2016
Publisher
Springer Berlin Heidelberg
Published in
European Journal of Pediatrics / Issue 4/2016
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI
https://doi.org/10.1007/s00431-015-2671-9

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