Acute Hemorrhagic Edema of Infancy Clinical Presentation

  • Author: Tina S Chen, MD; Chief Editor: William D James, MD   more...
 
Updated: Apr 4, 2012
 

History

Age of onset for acute hemorrhagic edema of infancy (AHEI) usually is 4-24 months but ranges from birth to 60 months. Clinical findings develop rapidly over 24-48 hours. Associated fever is common but tends to be low grade. Upper respiratory tract infection, gastroenteritis, drug intake (ie, antibiotics), or vaccination frequently precedes AHEI.[23, 24, 25, 26] Most cases occur in winter months.

Visceral and systemic involvement are uncommon but may include the following:

  • Joint pain
  • Abdominal pain, gastrointestinal tract bleeding, lethal intestinal complications (eg, intussusception)[18]
  • Scrotal pain and testicular torsion[19]

Spontaneous recovery usually occurs within 1-3 weeks,[27] with a reported duration of up to 35 days. Episodes of AHEI may recur.[8]

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Physical

The clinical picture for acute hemorrhagic edema of infancy (AHEI) is quite typical. Patients usually are nontoxic in appearance. Characteristic, rosette-, annular-, or targetoid-shaped purpuric lesions are symmetrically distributed primarily on the face, ears, and extremities.[28, 29]

Purpura may involve the scrotum.[19, 30] Lesions may begin as urticarial plaques and enlarge up to 5 cm in diameter. The borders are sharp. Purpura of the umbilicus can be mistaken for the Cullen sign (see the image below).

This toddler with acute hemorrhagic edema of infanThis toddler with acute hemorrhagic edema of infancy has a discoloration in the area of the umbilicus similar to that described as Cullen sign.

Mucosal involvement is rare but has been reported.

Acral edema involving the dorsum of the hands and feet frequently extends proximally up the extremities. Edema is usually nontender and may be asymmetric. The scalp may be involved. Joint and abdominal examinations are usually unremarkable. Note the images below.

The left leg in this patient with acute hemorrhagiThe left leg in this patient with acute hemorrhagic edema of infancy is markedly more edematous than the right leg. Note the concentric arcs of purpura on the patientNote the concentric arcs of purpura on the patient's arm. Despite the frightening appearance of purpura in tDespite the frightening appearance of purpura in these patients, they usually are in no significant distress.
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Causes

The cause of acute hemorrhagic edema of infancy (AHEI) is unknown. AHEI is likely an immune complex disorder; however, immune complexes have been demonstrated in only some cases.[20, 31]

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Contributor Information and Disclosures
Author

Tina S Chen, MD  Pediatric Dermatology Fellow, Rady Children's Hospital, Department of Dermatology, University of California, San Diego

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 College of Osteopathic Medicine; 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 Health Sciences Center, Paul L Foster 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, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania 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

William D James, MD  Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Mark Crowe, MD, to the development and writing of this article.

References

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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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