eMedicine Specialties > Dermatology > Pediatric Diseases

Acute Hemorrhagic Edema of Infancy: Differential Diagnoses & Workup

Author: Mark A Crowe, MD, Assistant Clinical Instructor, Department of Medicine, Division of Dermatology, University of Washington School of Medicine
Contributor Information and Disclosures

Updated: Sep 25, 2009

Differential Diagnoses

Acute Febrile Neutrophilic Dermatosis
Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis)
Dermatologic Manifestations of Hematologic Disease
Meningococcemia
Drug Eruptions
Oral Manifestations of Drug Reactions
Erythema Multiforme
Urticaria, Acute
Henoch-Schönlein Purpura (Anaphylactoid Purpura)

Other Problems to Be Considered

Child abuse

Workup

Laboratory Studies

  • In acute hemorrhagic edema of infancy (AHEI), routine laboratory tests are nondiagnostic.
  • Urinalysis results usually are normal.
  • Hematologic studies (eg, sedimentation rates, clotting studies) most often are normal.
  • Serum complement levels are normal.
  • Liver function test results may rarely be elevated.29
  • Serologic studies are unremarkable.

Imaging Studies

  • In acute hemorrhagic edema of infancy (AHEI), chest radiographs may demonstrate abnormal findings in cases with tuberculosis (2 reported cases of AHEI occurring with pulmonary tuberculosis).

Histologic Findings

Histologic changes in patients with acute hemorrhagic edema of infancy (AHEI) are those of a leukocytoclastic vasculitis. The characteristics of leukocytoclastic vasculitis are demonstrated as vascular changes with a cellular infiltrate containing many neutrophils. Only small blood vessels in the dermis are involved. Vessels show swelling of their endothelial cells and deposits of fibrin within and around their walls. The result is a smudgy appearance termed fibrinoid degeneration. The cellular infiltrate is predominantly perivascular and consists primarily of neutrophils. A characteristic feature of leukocytoclastic vasculitis is the presence of numerous scattered nuclear fragments, which is termed nuclear dust. Typically, extensive extravasation of erythrocytes is present.

Histopathologic findings in Henoch-Schönlein purpura (HSP) are identical to those seen in AHEI; however, the immunohistologic pattern found in AHEI is different from the pattern of HSP. Patients with HSP usually have IgA deposition. IgA deposition is demonstrable in only approximately one third of patients with AHEI.

Leukocytoclastic vasculitis and fibrinoid necrosi...

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).

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Leukocytoclastic vasculitis and fibrinoid necrosi...

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).


Direct immunofluorescence studies in patients with AHEI have revealed depositions of various immunoreactants, including fibrinogen, complement component C3, IgA, immunoglobulin G, immunoglobulin M, and immunoglobulin E (100%, 100%, 33%, 22%, 78%, and 33% of patients, respectively) 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%).

More on Acute Hemorrhagic Edema of Infancy

Overview: Acute Hemorrhagic Edema of Infancy
Differential Diagnoses & Workup: Acute Hemorrhagic Edema of Infancy
Treatment & Medication: Acute Hemorrhagic Edema of Infancy
Follow-up: Acute Hemorrhagic Edema of Infancy
Multimedia: Acute Hemorrhagic Edema of Infancy
References
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References

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

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Further Reading

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Keywords

acute hemorrhagic edema of infancy, AHEI, acute infantile hemorrhagic oedema, Finkelstein's disease, Seidlmayer syndrome, , cockade purpura with edema, postinfectious cockade purpura of early childhood, acute benign cutaneous leukocytoclastic vasculitis of infancy

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Contributor Information and Disclosures

Author

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.

Medical Editor

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.

Pharmacy Editor

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.

Managing Editor

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.

CME Editor

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 None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

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.

 
 
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