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Atopic dermatitis or hyper-IgE syndrome?

Nkiruka U Ohameje1, James W Loveless, Sarbjit S Saini

  • 1The Johns Hopkins Asthma and Allergy Center, Baltimore, Maryland 21224, USA.

Allergy and Asthma Proceedings
|August 18, 2006
PubMed
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Severe atopic dermatitis (AD) with recurrent infections and very high immunoglobulin E (IgE) may indicate immunodeficiency. Hyper-IgE syndrome (HIES) should be considered in such cases.

Area of Science:

  • Immunology
  • Dermatology
  • Infectious Diseases

Background:

  • Atopic dermatitis (AD) is a chronic inflammatory skin condition often associated with elevated immunoglobulin E (IgE) levels.
  • Recurrent infections can complicate AD, necessitating a thorough diagnostic workup.
  • Hyper-IgE syndrome (HIES) is a primary immunodeficiency characterized by extreme elevation of IgE.

Observation:

  • A case is presented of a patient with severe AD, recurrent infections, and markedly elevated IgE levels.
  • Deep-seated Staphylococcus aureus infections were noted, prompting consideration of underlying immunodeficiency.
  • The patient's IgE levels exceeded the typical range seen even in severe AD.

Findings:

  • The clinical presentation raised suspicion for HIES, a syndrome defined by elevated IgE, recurrent staphylococcal abscesses, and pneumonia with pneumatoceles.

Related Experiment Videos

  • While AD can have high IgE, levels in this severe case surpassed typical ranges.
  • The diagnostic criteria for HIES were considered in the context of the patient's symptoms.

    • Implications:

    • Rare, deep-seated Staphylococcus aureus infections in AD patients should prompt evaluation for immunodeficiency syndromes like HIES.
    • Accurate diagnosis of HIES is crucial for appropriate management and genetic counseling.
    • The efficacy and optimal dosing of anti-IgE therapy in AD and HIES remain areas requiring further investigation.