title: Acute hemorrhagic edema of young children: a prospective case series.
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Pediatric Department of Southern Switzerland, Bellinzona, Switzerland.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Ferrarini
authors.affiliation: Pediatric Department of Southern Switzerland, Bellinzona, Switzerland.
authors.forename: Cecilia
authors.initials: C
authors.lastname: Benetti
authors.affiliation: Pediatric Department of Southern Switzerland, Bellinzona, Switzerland.
authors.forename: Pietro
authors.initials: P
authors.lastname: Camozzi
authors.affiliation: Pediatric Department of Southern Switzerland, Bellinzona, Switzerland.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Ostini
authors.affiliation: Pediatric Department of Southern Switzerland, Bellinzona, Switzerland.
authors.forename: Giacomo D
authors.initials: GD
authors.lastname: Simonetti
authors.affiliation: Foundation IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Pediatric Emergency Department, Milan, Italy.
authors.forename: Gregorio P
authors.initials: GP
authors.lastname: Milani
authors.affiliation: Pediatric Department of Southern Switzerland, Bellinzona, Switzerland. mario.bianchetti@eoc.ch.
authors.forename: Mario G
authors.initials: MG
authors.lastname: Bianchetti
authors.affiliation: University Children's Hospital Berne, University of Berne, Berne, Switzerland.
authors.forename: Sebastiano A G
authors.initials: SA
authors.lastname: Lava
mesh_terms: D000208:Acute Disease; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004487:Edema / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011446:Prospective Studies; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000175:diagnosis*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic
keywords: Acute hemorrhagic edema of young children; Antineutrophil cytoplasmic autoantibodies; Child; Finkelstein–Seidlmayer disease; Leukocytoclastic vasculitis