Acute Hemorrhagic Edema of Infancy 

  • Author: Tina S Chen, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Oct 26, 2010
 

Background

Snow[1] first described acute hemorrhagic edema of infancy (AHEI) as a purely cutaneous variant of Henoch-Schönlein purpura (HSP) in the United States in 1913. Del Carril, Diaz Sobillo, and Vidal[2] described the condition in Argentina in 1936. Europeans have recognized Finkelstein's description of AHEI since his publication in 1938,[3] and, until recently, most reports of AHEI occurred in the European literature under the terms Finkelstein’s disease, Seidlmayer cockade purpura,[4] or purpura en cocarde avec oedema. Other names used for the disease include urticarial vasculitis of infancy and acute benign cutaneous leukocytoclastic vasculitis of infancy.[5]

More than 100 cases of AHEI have been published in medical literature worldwide. Although initially considered a variant of HSP, it is now considered a separate entity because of the infrequency of both visceral involvement and immunoglobulin A (IgA) skin depositions,[6, 5, 7, 8] as well as the better prognosis. AHEI is characterized by the triad of fever; edema; and rosette-, annular-, or targetoid-shaped purpura primarily over the face, ears, and extremities in a nontoxic infant (see the image below ).[9, 10, 11, 12] The cutaneous findings are dramatic both in appearance and rapidity of onset.

Large cockade (rosette or knot of ribbons), annulaLarge cockade (rosette or knot of ribbons), annular, or targetoid purpuric lesions found primarily on the face, ears, and extremities are characteristic of acute hemorrhagic edema of infancy.

Although once thought to be a variant of HSP, AHEI is now considered a separate entity. HSP usually presents with palpable purpura or petechiae associated with 1 or more symptoms, including abdominal pain, arthritis/arthralgias, and nephritis; however, any of these symptoms may be absent, which often leads to confusion in diagnosing the condition. The diagnosis may be particularly difficult to make when a patient presents with isolated symptoms, such as abdominal pain, without the typical rash. Scalp edema and/or scrotal swelling also may be seen in patients with HSP.

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Pathophysiology

The specific etiology of acute hemorrhagic edema of infancy (AHEI) is unknown. Eighty-four percent of reported cases were preceded by viral infections (acute upper respiratory tract infection, gastroenteritis), medication use (ie, antibiotics), and immunizations.[13, 14]

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Epidemiology

Frequency

United States

Acute hemorrhagic edema of infancy (AHEI) is uncommon in the United States. Specific frequency data have not been reported.

International

Acute hemorrhagic edema of infancy (AHEI) has been reported in countries throughout the world, although incidence is unknown.

Mortality/Morbidity

Acute hemorrhagic edema of infancy (AHEI) is usually benign and without sequelae, with spontaneous recovery occurring within 1-3 weeks. Rare reports have described complications such as arthritis, nephritis,[15, 16] abdominal pain, gastrointestinal tract bleeding, intussusception,[17] scrotal pain, and testicular torsion.[18] AHEI may recur, but this is uncommon. One case report describes an AHEI patient whose eruption resolved with unusual scarring.[14]

Race

No racial predilection has been described for acute hemorrhagic edema of infancy (AHEI).

Sex

Acute hemorrhagic edema of infancy (AHEI) is more common among male infants than among female infants[19] ; the male-to-female ratio is approximately 4.6:1.

Age

Age of onset for acute hemorrhagic edema of infancy (AHEI) usually is 2-60 months (median, 11 mo; mean, 13.75 mo).[19, 20] However, a congenital case of AHEI has been reported.[21]

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Proceed to Clinical Presentation
 
 
Contributor Information and Disclosures
Author

Tina S Chen, MD  Resident Physician, Department of Dermatology, University of California of Irvine, School of Medicine

Tina S Chen, MD is a member of the following medical societies: American Academy of Dermatology, California Society of Dermatology and Dermatologic Surgery, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Brandie J Metz, MD  Assistant Clinical Professor of Dermatology and Pediatrics, Chief of Pediatric Dermatology, University of California, Irvine, School of Medicine

Brandie J Metz, MD is a member of the following medical societies: American Academy of Dermatology, Society for Pediatric Dermatology, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Daniel J Hogan, MD  Clinical Professor of Internal Medicine (Dermatology), NOVA Southeastern University; Investigator, Hill Top Research, Florida Research Center

Daniel J Hogan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Contact Dermatitis Society, and Canadian Dermatology Association

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD  Associate Professor of Dermatology, Penn State University College of Medicine; Staff Dermatologist, Penn State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. Snow IM. Purpura, urticaria and angioneurotic edema of the hands and feet in a nursing baby. JAMA. 1913;61:18-19.

  2. Del Carril MJ, Diaz Sobillo I, Vidal J. Edema agudo hemorra´gico en un lactante. Prensa Med Argent. 1936;23:1719-22.

  3. Finkelstein H. Lehrbuch der Sauglingskrankheiten. 4th ed. Amsterdam: 1938:814-30.

  4. Seidlmayer H. Die Fruhinfantile postinfektiose Kokarde-Purpura. Z Kinderheilk. 1939;61:217-55.

  5. Saraclar Y, Tinaztepe K, Adalioglu G, Tuncer A. Acute hemorrhagic edema of infancy (AHEI)--a variant of Henoch-Schönlein purpura or a distinct clinical entity?. J Allergy Clin Immunol. Oct 1990;86(4 Pt 1):473-83. [Medline].

  6. Al-Sheyyab M, El-Shanti H, Ajlouni S, Sawalha D, Daoud A. The clinical spectrum of Henoch-Schönlein purpura in infants and young children. Eur J Pediatr. Dec 1995;154(12):969-72. [Medline].

  7. Shah D, Goraya JS, Poddar B, Parmar VR. Acute infantile hemorrhagic edema and Henoch-Schonlein purpura overlap in a child. Pediatr Dermatol. Jan-Feb 2002;19(1):92-3. [Medline].

  8. Legrain V, Lejean S, Taieb A, Guillard JM, Battin J, Maleville J. Infantile acute hemorrhagic edema of the skin: study of ten cases. J Am Acad Dermatol. Jan 1991;24(1):17-22. [Medline].

  9. Crowe MA, Jonas PP. Acute hemorrhagic edema of infancy. Cutis. Aug 1998;62(2):65-6. [Medline].

  10. Karremann M, Jordan AJ, Bell N, Witsch M, Durken M. Acute hemorrhagic edema of infancy: report of 4 cases and review of the current literature. Clin Pediatr (Phila). Apr 2009;48(3):323-6. [Medline].

  11. Michael DJ. Acute hemorrhagic edema of infancy. Dermatol Online J. Sep 8 2006;12(5):10. [Medline].

  12. Sites LY, Woodmansee CS, Wilkin NK, Hanson JW, Skinner RB Jr, Shimek CM. Acute hemorrhagic edema of infancy: case reports and a review of the literature. Cutis. Nov 2008;82(5):320-4. [Medline].

  13. Cox NH. Seidlmayer's syndrome: postinfectious cockade purpura of early childhood. J Am Acad Dermatol. Feb 1992;26(2 Pt 1):275. [Medline].

  14. AlSufyani MA. Acute hemorrhagic edema of infancy: unusual scarring and review of the English language literature. Int J Dermatol. Jun 2009;48(6):617-22. [Medline].

  15. Allen DM, Diamond LK, Howell DA. Anaphylactoid purpura in children (Schonlein-Henoch syndrome): review with a follow-up of the renal complications. AMA J Dis Child. Jun 1960;99:833-54. [Medline].

  16. Watanabe T, Sato Y. Renal involvement and hypocomplementemia in a patient with acute hemorrhagic edema of infancy. Pediatr Nephrol. Nov 2007;22(11):1979-81. [Medline].

  17. Yu JE, Mancini AJ, Miller ML. Intussusception in an infant with acute hemorrhagic edema of infancy. Pediatr Dermatol. Jan-Feb 2007;24(1):61-4. [Medline].

  18. Medrano San Ildefonso M, Bruscas Izu C, Ferrer Lozano M, Pastor Mouron I. [Scrotal involvement in Schönlein-Henoch purpura]. An Esp Pediatr. Jan 1998;48(1):102-3. [Medline].

  19. Fiore E, Rizzi M, Ragazzi M, et al. Acute hemorrhagic edema of young children (cockade purpura and edema): a case series and systematic review. J Am Acad Dermatol. Oct 2008;59(4):684-95. [Medline].

  20. Cunningham BB, Eramo L, Caro W. Acute hemorrhagic edema of childhood present at birth. Pediatr Dermatol. Jan-Feb 1999;16(1):68. [Medline].

  21. Cunningham BB, Caro WA, Eramo LR. Neonatal acute hemorrhagic edema of childhood: case report and review of the English-language literature. Pediatr Dermatol. Jan-Feb 1996;13(1):39-44. [Medline].

  22. Di Lernia V, Lombardi M, Lo Scocco G. Infantile acute hemorrhagic edema and rotavirus infection. Pediatr Dermatol. Sep-Oct 2004;21(5):548-50. [Medline].

  23. Garty BZ, Pollak U, Scheuerman O, Marcus N, Hoffer V. Acute hemorrhagic edema of infancy associated with herpes simplex type 1 stomatitis. Pediatr Dermatol. Jul-Aug 2006;23(4):361-4. [Medline].

  24. Jeannoel P, Fabre M, Payen C, Bost M. [Acute hemorrhagic edema in infants: role of adenoviruses? Apropos of a case]. Pediatrie. Oct-Nov 1985;40(7):557-60. [Medline].

  25. Morrison RR, Saulsbury FT. Acute hemorrhagic edema of infancy associated with pneumococcal bacteremia. Pediatr Infect Dis J. Sep 1999;18(9):832-3. [Medline].

  26. Krause I, Lazarov A, Rachmel A, et al. Acute haemorrhagic oedema of infancy, a benign variant of leucocytoclastic vasculitis. Acta Paediatr. Jan 1996;85(1):114-7. [Medline].

  27. Macea JM, Santi CG, Sotto MN, Caputo R. Multiple erythematous plaques on a child. Acute hemorrhagic edema (AHE) of infancy. Arch Dermatol. Apr 2003;139(4):531-6. [Medline].

  28. McDougall CM, Ismail SK, Ormerod A. Acute haemorrhagic oedema of infancy. Arch Dis Child. Mar 2005;90(3):316. [Medline].

  29. Khan AU, Williams TH, Malek RS. Acute scrotal swelling in Henoch-Schönlein syndrome. Urology. Aug 1977;10(2):139-41. [Medline].

  30. Goraya JS, Kaur S. Acute infantile hemorrhagic edema and Henoch-Schonlein purpura: is IgA the missing link?. J Am Acad Dermatol. Nov 2002;47(5):801; author reply 801-2. [Medline].

  31. Kumar R, Mittal K, Rawal M, Kumar S. Acute hemorrhagic edema of infancy. Indian Pediatr. Dec 2008;45(12):1002-3. [Medline].

  32. Jain G, Patel A. Acute hemorrhagic edema of infancy. Indian Pediatr. Oct 2008;45(10):866-7. [Medline].

  33. Obeid M, Haley J, Crews J, Parhizgar R, Johnson L, Camp T. Acute hemorrhagic edema of infancy with abdominal pain and elevated transaminases. Pediatr Dermatol. Nov-Dec 2008;25(6):640-1. [Medline].

 
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Large cockade (rosette or knot of ribbons), annular, or targetoid purpuric lesions found primarily on the face, ears, and extremities are characteristic of acute hemorrhagic edema of infancy.
The left leg in this patient with acute hemorrhagic edema of infancy is markedly more edematous than the right leg.
Leukocytoclastic vasculitis and fibrinoid necrosis is seen in patients with acute hemorrhagic edema of infancy. This histologic pattern also is seen in Henoch-Schönlein purpura, although patients with Henoch-Schönlein purpura usually have immunoglobulin A deposition, and immunoglobulin A deposition is demonstrable in only approximately one third of patients with acute hemorrhagic edema of infancy (hematoxylin and eosin, magnification X40).
This toddler with acute hemorrhagic edema of infancy has a discoloration in the area of the umbilicus similar to that described as Cullen sign.
Note the concentric arcs of purpura on the patient's arm.
Despite the frightening appearance of purpura in these patients, they usually are in no significant distress.
 
 
 
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