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Related Concept Videos

Hypersensitivities01:30

Hypersensitivities

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Hypersensitivity, also known as a hypersensitivity reaction or allergic reaction, is a condition where the body's immune system reacts abnormally to a foreign substance. Such substances, that cause hypersensitivity are referred to as an allergen, could be something typically harmless to most people, like pollen or certain foods.
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Hypersensitivity Reactions: Immune-Complex Reactions01:19

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Type III hypersensitivity reactions occur when antigen–antibody complexes form and activate the complement system. Normally, these complexes help the clearance of antigens by phagocytes and red blood cells. However, when large numbers of immune complexes are present, they can deposit in tissues—particularly in the walls of blood vessels—leading to inflammation and tissue injury. These deposits trigger complement activation and neutrophil recruitment, resulting in serum...
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Irritable Bowel Syndrome I: Introduction01:17

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Irritable Bowel Syndrome II: Clinical Features and Diagnostic Evaluation
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Related Experiment Video

Updated: Mar 10, 2026

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
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Non-syndromic hyper-IgE in children: A practical approach.

Riccardo Castagnoli1,2, Luca Pecoraro3, Carla Mastrorilli4

  • 1Pediatric Unit, Department of Clinical, Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Pavia, 27100, Italy.

The World Allergy Organization Journal
|March 9, 2026
PubMed
Summary

Elevated serum IgE in children can indicate common allergies or rare inborn errors of immunity (IEI). This review offers a diagnostic framework to differentiate these conditions, improving accuracy and early intervention for hyper-IgE syndromes.

Keywords:
Elevated IgE levelsHyper-IgEInborn errors of immunityallergyatopyparasitic infections

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Area of Science:

  • Pediatric Immunology
  • Clinical Diagnostics

Background:

  • Hyper-immunoglobulin E (Hyper-IgE) is defined by serum IgE >2000 IU/mL, posing a diagnostic challenge in pediatrics.
  • Elevated IgE can stem from atopic conditions, infections, or rarer inborn errors of immunity (IEI), specifically hyper-IgE syndrome (HIES).
  • Differentiating common causes from HIES is crucial for early-onset, severe, or treatment-resistant cases.

Purpose of the Study:

  • To provide a practical, structured framework for clinicians managing non-syndromic hyper-IgE in children.
  • To guide diagnostic reasoning through a systematic approach incorporating clinical history, examination, and investigations.
  • To facilitate timely referral for immunologic or genetic evaluation when IEI is suspected.

Main Methods:

  • Review of diverse etiologies of elevated serum IgE, including allergic diseases, infections, inflammatory disorders, malignancies, drug reactions, and environmental factors.
  • Proposal of a stepwise diagnostic algorithm to prioritize common causes and identify red flags for rare conditions.
  • Emphasis on detailed clinical history, physical examination, and targeted investigations.

Main Results:

  • A broad differential diagnosis exists for hyper-IgE, necessitating a systematic approach.
  • The proposed algorithm aids in distinguishing common conditions from IEI or other rare causes.
  • Increased awareness and a structured approach can improve diagnostic accuracy and reduce morbidity.

Conclusions:

  • A systematic diagnostic strategy is critical for managing hyper-IgE in children.
  • Early identification of IEI or rare conditions through a structured approach leads to better outcomes.
  • Further research is needed to refine diagnostic strategies and long-term follow-up protocols.