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Acute Hemorrhagic Edema of Infancy Workup

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

Laboratory Studies

In acute hemorrhagic edema of infancy (AHEI), routine laboratory tests are nondiagnostic, although the following may be performed to exclude other conditions:

  • Urinalysis results usually are normal.
  • Hematologic studies (eg, blood cell counts, clotting studies) are often normal, although leukocytosis and thrombocytosis may be found.[35]
  • Erythrocyte sedimentation rates may be normal or elevated.[15, 35, 36]
  • Serum complement levels are normal.
  • Liver function test results may rarely be elevated.[37]
  • Serologic studies are unremarkable.
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Imaging Studies

No imaging studies are necessary in the workup for acute hemorrhagic edema of infancy (AHEI).

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Histologic Findings

Acute hemorrhagic edema of infancy (AHEI) is an immune complex-mediated leukocytoclastic vasculitis, usually limited to the small blood vessels of the dermis.[29] The characteristics of leukocytoclastic vasculitis are demonstrated as vascular changes with a perivascular infiltrate consisting primarily of neutrophils; nuclear dust is commonly seen. Vessels show swelling of their endothelial cells and deposits of fibrin within and around their walls, resulting in a “smudgy” appearance termed fibrinoid degeneration. Typically, extensive extravasation of erythrocytes is present.

Direct immunofluorescence studies in patients with AHEI reveal depositions of various immunoreactants, including fibrinogen, immunoglobulin A (IgA), immunoglobulin G, immunoglobulin M, immunoglobulin E, and complement C3 deposition[5] in the wall and around small vessels. Similar deposition of C1q complement also was present in 3 infants in whom C1q complement could be studied (100%). Of the immunoglobulins, IgA deposition is the most common, although this finding occurs in only 10-35% of AHEI cases,[35] thus helping to differentiate AHEI from Henoch-Schönlein purpura.

Note the image below.

Leukocytoclastic vasculitis and fibrinoid necrosisLeukocytoclastic 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).
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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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