Skip to main content

Advertisement

SpringerLink
Log in

Accurate diagnosis of acute hemorrhagic edema of infancy: a French multicenter observational study

  • RESEARCH
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

The purpose of the study is to highlight clinical signs that are either suggestive of or against the diagnosis of AHEI to improve diagnosis and management. The medical records of children under 3 years old diagnosed with AHEI were retrospectively reviewed. Clinical data and photographs were reviewed by three independent experts, and the cases were classified as probable, doubtful, or unclear AHEI. Of the 69 cases of children diagnosed with AHEI included in 22 centers, 40 were classified as probable, 22 as doubtful, and 7 as unclear. The median age of patients with probable AHEI was 11 months [IQR 9–15], and they were in overall good condition (n = 33/40, 82.5%). The morphology of the purpura was targetoid in 75% of cases (n = 30/40) and ecchymotic in 70% of cases (n = 28/40) and affected mostly the legs (n = 39/40, 97%), the arms (n = 34/40, 85%), and the face (n = 33/40, 82.5%). Edema was observed in 95% of cases and affected mostly the hands (n = 36/38, 95%) and feet (n = 28/38, 74%). Pruritus was absent in all patients with probable AHEI and described for 6/21 with doubtful AHEI (29%). AHEI was the original diagnosis in only 24 patients (n = 24/40, 60%). The major differential diagnoses were purpura fulminans and urticaria multiforme.

  Conclusion: AHEI, which the diagnosis is made on clinical findings, is often misdiagnosed. Purpuric lesions localized on the face/ears, arms/forearms, and thighs/legs with edema of the hands without pruritus in a young child with a good overall condition are highly suggestive of AHEI.

What is Known:

•Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic vasculitis affecting children under 3 years old.

•Appropriate diagnosis is important to distinguish this benign disease from more serious diseases to avoid investigations and treatments, iatrogenic harm and unnecessary follow-up.

What is New:

•AHEI is an uncommon disorder often misdiagnosed by pediatricians and dermatologists.

•Purpuric lesions localized on the face/ears, arms/forearms, and thighs/legs with edema of the hands without pruritus in an infant with a good overall condition are highly suggestive of AHEI.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

Data availability

Data supporting the study findings are available from the corresponding author on reasonable request.

Abbreviations

AHEI:

Acute hemorrhagic edema of infancy

IgAV:

IgA vasculitis

References

  1. Johnson EF, Wetter DA, Lehman JS et al (2017) Leukocytoclastic vasculitis in children: clinical characteristics, subtypes, causes and direct immunofluorescence findings of 56 biopsy-confirmed cases. J Eur Acad Dermatol Venereol 31:544–549

    Article  CAS  PubMed  Google Scholar 

  2. Ting TV (2014) Diagnosis and management of cutaneous vasculitis in children. Pediatr Clin North Am 61:321–346

    Article  PubMed  Google Scholar 

  3. Fiore E, Rizzi M, Ragazzi M et al (2008) Acute hemorrhagic edema of young children (cockade purpura and edema): a case series and systematic review. J Am Acad Dermatol 59:684–695

    Article  PubMed  Google Scholar 

  4. Leung AKC, Leong KF, Lam JM (2020) Acute hemorrhagic edema of infancy: a diagnostic challenge for the general pediatrician. Curr Pediatr Rev 16:285–293

    Article  PubMed  Google Scholar 

  5. Fiore E, Rizzi M, Simonetti GD et al (2011) Acute hemorrhagic edema of young children: a concise narrative review. Eur J Pediatr 170:1507–1511

    Article  PubMed  Google Scholar 

  6. Parker L, Shahar-Nissan K, Ashkenazi-Hoffnung L et al (2017) Acute hemorrhagic edema of infancy: the experience of a large tertiary pediatric center in Israel. World J Pediatr 13:341–345

    Article  CAS  PubMed  Google Scholar 

  7. Homme JL, Block JM (2016) Acute hemorrhagic edema of infancy and common mimics. Am J Emerg Med 34:936.e3–6

    Article  PubMed  Google Scholar 

  8. Saraclar Y, Tinaztepe K, Adalioglu G, Tuncer A (1990) Acute hemorrhagic edema of infancy (AHEI)–a variant of Henoch-Schonlein purpura or a distinct clinical entity? J Allergy Clin Immunol 86:473–483

    Article  CAS  PubMed  Google Scholar 

  9. Ince E, Mumcu Y, Suskan E et al (1995) Infantile acute hemorrhagic edema: a variant of leukocytoclastic vasculitis. Pediatr Dermatol 12:224–227

    Article  CAS  PubMed  Google Scholar 

  10. Karremann M, Jordan AJ, Bell N et al (2009) Acute hemorrhagic edema of infancy: report of 4 cases and review of the current literature. Clin Pediatr (Phila) 48:323–326

    Article  PubMed  Google Scholar 

  11. Moradinejad MH, Entezari P, Mahjoub F, Ziaee V (2011) Acute hemorrhagic edema of infancy; a report of five Iranian infants and review of the literature. Iran J Pediatr 21:107–112

    PubMed  PubMed Central  Google Scholar 

  12. Caksen H, Odabas D, Kosem M et al (2002) Report of eight infants with acute infantile hemorrhagic edema and review of the literature. J Dermatol 29:290–295

    Article  PubMed  Google Scholar 

  13. Legrain V, Lejean S, Taieb A et al (1991) Infantile acute hemorrhagic edema of the skin: study of ten cases. J Am Acad Dermatol 24:17–22

    Article  CAS  PubMed  Google Scholar 

  14. Poyrazoglu HM, Per H, Gunduz Z et al (2003) Acute hemorrhagic edema of infancy. Pediatr Int 45:697–700

    Article  PubMed  Google Scholar 

  15. Emerich PS, Prebianchi PA, Motta LL et al (2011) Acute hemorrhagic edema of infancy: report of three cases. An Bras Dermatol 86:1181–1184

    Article  PubMed  Google Scholar 

  16. Suehiro RM, Soares BS, Eisencraft AP et al (2007) Acute hemorrhagic edema of childhood. Turk J Pediatr 49:189–192

    PubMed  Google Scholar 

  17. Ferrarini A, Benetti C, Camozzi P et al (2016) Acute hemorrhagic edema of young children: a prospective case series. Eur J Pediatr 175:557–561

    Article  PubMed  Google Scholar 

  18. Dubin BA, Bronson DM, Eng AM (1990) Acute hemorrhagic edema of childhood: an unusual variant of leukocytoclastic vasculitis. J Am Acad Dermatol 23:347–350

    Article  CAS  PubMed  Google Scholar 

  19. Cunha DF, Darcie AL, Benevides GN et al (2015) Acute hemorrhagic edema of infancy: an unusual diagnosis for the general pediatrician. Autops Case Rep 5:37–41

    Article  PubMed  PubMed Central  Google Scholar 

  20. Ceci M, Conrieri M, Raffaldi I et al (2018) Acute hemorrhagic edema of infancy: still a challenge for the pediatrician. Pediatr Emerg Care 34:e28–e29

    Article  PubMed  Google Scholar 

  21. Robl R, Robl M, Marinoni LP et al (2014) Target-shaped edematous purple lesions: is it child abuse? Arch Dis Child 99:44–45

    Article  PubMed  Google Scholar 

  22. Tamayo-Sanchez L, Ruiz-Maldonado R, Laterza A (1997) Acute annular urticaria in infants and children. Pediatr Dermatol 14:231–234

    Article  CAS  PubMed  Google Scholar 

  23. Krause I, Lazarov A, Rachmel A et al (1996) Acute haemorrhagic oedema of infancy, a benign variant of leucocytoclastic vasculitis. Acta Paediatr 85:114–117

    Article  CAS  PubMed  Google Scholar 

  24. Bronz G, Betti C, Rinoldi PO et al (2022) Infections or vaccines associated with Finkelstein-Seidlmayer vasculitis: systematic review. Clin Rev Allergy Immunol 63:490–498

    Article  PubMed  PubMed Central  Google Scholar 

  25. Rinoldi PO, Bronz G, Ferrarini A et al (2021) Acute hemorrhagic edema: uncommon features. J Am Acad Dermatol 85:1620–1621

    Article  PubMed  Google Scholar 

  26. Gao PR, Yen H, Chen WT (2020) Acute hemorrhagic edema of infancy. CMAJ 192:E1309

  27. Gattorno M, Picco P, Vignola S et al (1999) Brother and sister with different vasculitides. Lancet 353:728

    Article  CAS  PubMed  Google Scholar 

  28. Piram M, Bodemer C, Kone-Paut I (2009) Signes dermatologiques des vascularites de l’enfant. Arch Pediatr 16:526–528

    Article  CAS  PubMed  Google Scholar 

  29. Chalmers E, Cooper P, Forman K et al (2011) Purpura fulminans: recognition, diagnosis and management. Arch Dis Child 96:1066–1071

    Article  CAS  PubMed  Google Scholar 

  30. Bronz G, Gianini J, Passi AG et al (2023) Autoimmune markers and vascular immune deposits in Finkelstein-Seidlmayer vasculitis: systematic literature review. J Autoimmun 136:103002

    Article  CAS  PubMed  Google Scholar 

  31. Murgu A, Mihaila D, Cozma L, Chiforeanu AM (2012) Indications and limitations of histopathological skin investigation of Henoch-Schonlein purpura in children. Rom J Morphol Embryol 53:769–773

    PubMed  Google Scholar 

  32. Adcock DM, Hicks MJ (1990) Dermatopathology of skin necrosis associated with purpura fulminans. Semin Thromb Hemost 16:283–292

    Article  CAS  PubMed  Google Scholar 

  33. Emer JJ, Bernardo SG, Kovalerchik O, Ahmad M (2013) Urticaria multiforme J Clin Aesthet Dermatol 6:34–39

    PubMed  Google Scholar 

  34. Arend SM, Lavrijsen AP, Kuijken I et al (2006) Prospective controlled study of the diagnostic value of skin biopsy in patients with presumed meningococcal disease. Eur J Clin Microbiol Infect Dis 25:643–649

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

We thank Greta Gourier (CH de Cornouaille, Quimper), François Arditty (CH Versailles), Xavier Balguerie (Hôpital Charles Nicolle, Rouen), Romain Longuet (CHU Rennes), Karen Milcent (CHU Antoine Béclère, Clamart), and Catherine Eschard (Hôpital Robert Debré, Reims) for their participation in the data collection. We also thank the Research Group of the French Society of Pediatric Dermatology for its contribution.

Author information

Authors and Affiliations

  1. Department of Dermatology and Reference Center for Rare Diseases and Vascular Malformations (MAGEC), Unit of Pediatric Dermatology, CHRU Tours, Tours, France

    Sophie Leducq & Annabel Maruani

  2. Universities of Tours and Nantes, INSERM 1246–SPHERE, , Tours, France

    Sophie Leducq & Annabel Maruani

  3. Department of Dermatology and Reference Center for Genodermatoses and Rare Skin Diseases (MAGEC), APHP, Paris University, Necker-Enfants Malades Hospital, Paris Centre University, Imagine Institute, Paris, France

    Christine Bodemer & Olivia Boccara

  4. Department of Pediatrics, CHI Créteil, Créteil, France

    Sandra Biscardi

  5. Department of Pediatrics, CHRU Tours, Tours, France

    Marie-France Chinazzo

  6. Department of Dermatology, Hôpital Victor Dupouy, Argenteuil, France

    Emmanuel Mahé

  7. Department of Dermatology, Quimper, Quimper, CH, France

    Patrice Plantin

  8. Pathology Department, Necker-Enfants Malades Hospital, APHP, Paris, France

    Sylvie Fraitag

  9. Department of Dermatology, CHU Toulouse, Toulouse, France

    Juliette Mazereeuw-Hautier

  10. Department of Dermatology, University Hospital of Nice, Nice, France

    Christine Chiaverini

  11. Paediatric Onco-Haematology, Brabois Hospital, University Hospital of Nancy, Vandoeuvre-Lès-Nancy, France

    Irene Lemelle

  12. Department of Dermatology, University Hospital of Montpellier, Montpellier, France

    Didier Bessis

  13. Department of General Pediatrics, Robert Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France

    Emmanuelle Bourrat

  14. Department of Dermatology, Hôpital Timone, Aix-Marseille Université, Marseille, France

    Stéphanie Mallet

  15. Department of Dermatology and Genetics of Developmental Anomalies, UMR Inserm 1231, François-Mitterrand University Hospital, 14 Rue Paul Gaffarel, BP 77908, 21079, Dijon, France

    Bertille Bonniaud

  16. Department of Pediatrics, CHU Rouen, Rouen, France

    Martine Grall-Lerosey

  17. Department of Dermatology, CHU Angers, Angers, France

    Ludovic Martin

  18. Pediatric Dermatology Unit, Hôpital Pellegrin-Enfants, CHU de Bordeaux, France

    Franck Boralevi

  19. Department of Pediatrics, CHU Sainte Justine Research Centre, Sainte Justine University Hospital, University of Montreal, Montreal, QC, Canada

    Maryam Piram

Authors
  1. Sophie Leducq
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Annabel Maruani
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Christine Bodemer
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Sandra Biscardi
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Olivia Boccara
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Marie-France Chinazzo
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Emmanuel Mahé
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Patrice Plantin
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Sylvie Fraitag
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Juliette Mazereeuw-Hautier
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Christine Chiaverini
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Irene Lemelle
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Didier Bessis
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Emmanuelle Bourrat
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Stéphanie Mallet
    View author publications

    You can also search for this author in PubMed Google Scholar

  16. Bertille Bonniaud
    View author publications

    You can also search for this author in PubMed Google Scholar

  17. Martine Grall-Lerosey
    View author publications

    You can also search for this author in PubMed Google Scholar

  18. Ludovic Martin
    View author publications

    You can also search for this author in PubMed Google Scholar

  19. Franck Boralevi
    View author publications

    You can also search for this author in PubMed Google Scholar

  20. Maryam Piram
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by Sophie Leducq, Annabel Maruani, Maryam Piram. The first draft of the manuscript was written by Sophie Leducq, Annabel Maruani and Maryam Piram and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Sophie Leducq.

Ethics declarations

Ethics approval

This non-interventional research (retrospective research on previously collected data, other than genetic data) was conducted in accordance with the French Data Protection Agency.

Consent for publication

The authors affirm that human research participants provided informed consent for publication of the images in Fig. 3.

Competing interests

The authors declare no competing interests.

Additional information

Communicated by Tobias Tenenbaum

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary Information

Below is the link to the electronic supplementary material.

Supplementary file1 (DOCX 20 KB)

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Leducq, S., Maruani, A., Bodemer, C. et al. Accurate diagnosis of acute hemorrhagic edema of infancy: a French multicenter observational study. Eur J Pediatr 182, 4133–4141 (2023). https://doi.org/10.1007/s00431-023-05098-7

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00431-023-05098-7

Keywords

Search

Navigation