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Acute Hemorrhagic Edema of Infancy Treatment & Management

  • Author: Donald Shenenberger, MD, FAAD, FAAFP; Chief Editor: William D James, MD  more...
 
Updated: May 02, 2016
 

Medical Care

No effective therapy exists for acute hemorrhagic edema of infancy (AHEI). The use of steroids and antihistamines has been controversial, and they do not appear to alter the disease course. However, systemic corticosteroids may be used to ameliorate the acute manifestations of the disease.[27] Treatment is symptomatic; discontinue antibiotics after obtaining negative culture results.

Inpatient care is not usually required unless the diagnosis of acute hemorrhagic edema of infancy (AHEI) is in doubt. If meningococcemia or another significant condition remains in the differential diagnosis, patients may require monitoring or therapy as appropriate for those disorders.

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Consultations

Consult a dermatologist if the diagnosis of acute hemorrhagic edema of infancy (AHEI) is in doubt. Additionally, consult a gastroenterologist or nephrologist if significant abdominal symptoms or renal involvement is noted.

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Diet

Acute hemorrhagic edema of infancy (AHEI) patients usually are nontoxic in appearance. Although visceral involvement is rare, maintain a relatively bland diet with plenty of fluids to maintain hydration.

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Activity

No particular restrictions in activity are required for acute hemorrhagic edema of infancy (AHEI).

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Complications

Rare reports have described complications such as arthritis, nephritis,[19, 20] abdominal pain, gastrointestinal tract bleeding, intussusception,[21] scrotal pain, compartment syndrome,[28] and testicular torsion.[22]

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Prevention

No known method exists for preventing acute hemorrhagic edema of infancy (AHEI) or recurrences of the condition.

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Long-Term Monitoring

Treatment for acute hemorrhagic edema of infancy (AHEI) is symptomatic. Monitor patients for abdominal or renal involvement, which, although rare, has been reported.

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Contributor Information and Disclosures
Author

Donald Shenenberger, MD, FAAD, FAAFP Virginia Dermatology and Skin Cancer Center; Assistant Professor of Dermatology, Eastern Virginia Medical School

Donald Shenenberger, MD, FAAD, FAAFP is a member of the following medical societies: American Academy of Dermatology, American Academy of Family Physicians, Association of Military Dermatologists, Uniformed Services Academy of Family Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

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, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

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, Society for Investigative Dermatology, Association of Professors of Dermatology, North American Hair Research Society

Disclosure: Nothing to disclose.

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, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

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, Canadian Dermatology Association

Disclosure: Nothing to disclose.

Tina S Chen Twu, MD Pediatric Dermatologist/Dermatologist, Sharp Rees-Stealy Medical Group, San Diego, CA

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

Disclosure: Nothing to disclose.

Acknowledgements

Mark A Crowe, MD Assistant Clinical Instructor, Department of Medicine, Division of Dermatology, University of Washington School of Medicine

Mark A Crowe, MD is a member of the following medical societies: American Academy of Dermatology and North American Clinical Dermatologic Society

Disclosure: Nothing to disclose.

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.

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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