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Acute hemorrhagic edema of young children: a prospective case series

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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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Authors’ contributions

Alessandra Ferrarini, Mario G. Bianchetti, and Sebastiano A. G. Lava participated in the study concept and design. Cecilia Benetti, Pietro Camozzi, Alessandro Ostini, Giacomo D. Simonetti, and Gregorio P. Milani participated in the acquisition, analysis, and interpretation of data. Giacomo D. Simonetti and Mario G. Bianchetti participated in the statistical analysis. Alessandra Ferrarini, Pietro Camozzi, Alessandro Ostini, and Gregorio P. Milani participated in the drafting of the manuscript. Cecilia Benetti, Giacomo D. Simonetti, Gregorio P. Milani, Mario G. Bianchetti, and Sebastiano A. G. Lava participated in the critical revision of the manuscript.

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Author notes

  1. Gregorio P. Milani

    Present address: Foundation IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Pediatric Emergency Department, Milan, Italy

  2. Sebastiano A. G. Lava

    Present address: University Children’s Hospital Berne, University of Berne, Berne, Switzerland

Authors and Affiliations

  1. Pediatric Department of Southern Switzerland, Bellinzona, Switzerland

    Alessandra Ferrarini, Cecilia Benetti, Pietro Camozzi, Alessandro Ostini, Giacomo D. Simonetti & Mario G. Bianchetti

Authors
  1. Alessandra Ferrarini
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  2. Cecilia Benetti
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  3. Pietro Camozzi
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  4. Alessandro Ostini
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  5. Giacomo D. Simonetti
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  6. Gregorio P. Milani
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  7. Mario G. Bianchetti
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  8. Sebastiano A. G. Lava
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Corresponding author

Correspondence to Mario G. Bianchetti.

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Conflict of interest

The authors declare that they have no competing interests.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards and with the 1964 Helsinki declaration and its later amendments.

Informed consent

Informed consent was obtained from all participants.

Additional information

Communicated by David Nadal

Alessandra Ferrarini and Cecilia Benetti contributed equally to this work.

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Ferrarini, A., Benetti, C., Camozzi, P. et al. Acute hemorrhagic edema of young children: a prospective case series. Eur J Pediatr 175, 557–561 (2016). https://doi.org/10.1007/s00431-015-2671-9

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  • DOI: https://doi.org/10.1007/s00431-015-2671-9

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