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Updated: Jun 13, 2026
07:10
Application of Biochip Microfluidic Technology to Detect Serum Allergen-specific Immunoglobulin E (sIgE)
Published on: April 21, 2019
Hyper IgE syndrome: review and future directions
Alexandra F Freeman1, Steven M Holland
1National Institute of Allergy and Infectious Disease, National Institutes of Health, Building 10, CRC B3-4141, MSC 1684, Bethesda, MD 20892-1684, USA. freemaal@mail.nih.gov
Expert Review of Clinical Immunology
|May 19, 2010
Summary
Hyperimmunoglobulin E syndrome (HIES), or Job's syndrome, is a primary immunodeficiency with eczema, infections, and high IgE. Its genetic cause is unknown, impacting multiple body systems.
Area of Science:
- Immunology
- Genetics
- Rheumatology
Background:
- Hyperimmunoglobulin E syndrome (HIES), also known as Job's syndrome, is a primary immunodeficiency.
- Characterized by eczema, recurrent infections, and markedly elevated serum immunoglobulin E (IgE).
- Now recognized as a multisystem disorder affecting skeletal and morphological features.
Purpose of the Study:
- To review the clinical and immunological characteristics of HIES.
- To highlight the multisystemic nature of HIES.
- To emphasize the need for identifying the genetic etiology of HIES.
Main Methods:
- Literature review of HIES.
- Analysis of clinical manifestations.
- Discussion of current therapeutic approaches.
Main Results:
- HIES presents with recurrent staphylococcal abscesses, pneumonias, and pneumatocoeles.
- Skeletal features include osteopenia, scoliosis, fractures, and retained primary teeth.
- Therapy involves skin care, antimicrobial prophylaxis, and infection management.
Conclusions:
- The genetic basis of HIES remains elusive.
- Identifying the genetic cause will enable targeted therapies.
- Understanding HIES genetics advances knowledge of immunologic and connective tissue interactions.
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