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Published on: November 3, 2023
Acute hemorrhagic edema of young children: a prospective case series
Alessandra Ferrarini1, Cecilia Benetti1, Pietro Camozzi1
1Pediatric Department of Southern Switzerland, Bellinzona, Switzerland.
Insights
Acute hemorrhagic edema of young children is a rare, benign vasculitis. This prospective study confirms its characteristic features, including targetoid lesions and non-pitting edema, with complete resolution in affected children.
Area of Science:
- Pediatrics
- Dermatology
- Rheumatology
Background:
- Acute hemorrhagic edema of young children (AHEC) is a rare leukocytoclastic vasculitis.
- Previous reports are limited to small retrospective series or case studies.
Purpose of the Study:
- To prospectively evaluate the clinical features and outcomes of AHEC in children.
- To confirm the distinctive diagnostic tetrad of AHEC.
Main Methods:
- Prospective observational study of 16 children (5-28 months) diagnosed with AHEC.
- Clinical assessment included lesion characteristics, systemic involvement, and laboratory findings.
- Follow-up for resolution and recurrence.
Main Results:
- All patients presented with targetoid lesions, non-pitting edema, and were generally well-appearing with minimal fever.
- No evidence of mucous membrane, articular, abdominal, or kidney involvement was observed.
- Complete resolution occurred within 4 weeks in all cases, with no recurrences or familial cases noted.
Conclusions:
- This is the first prospective evaluation of AHEC, highlighting its benign nature.
- Key features include a well-appearing child, stable targetoid lesions, non-pitting edema, and absence of extracutaneous manifestations.
- Antineutrophil cytoplasmic autoantibodies are not implicated in the pathogenesis of AHEC.
Unlabelled:
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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