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Hyperimmunoglobulin E syndrome - Overview

Alternative Names

Job syndrome; Hyper IgE syndrome

Definition of Hyperimmunoglobulin E syndrome:

Hyperimmunoglobulin E syndrome is a rare, inherited disease that causes problems with the skin, sinuses, lungs, bones, and teeth.

Causes, incidence, and risk factors:

Hyperimmunoglobulin E syndrome is also called Job syndrome, after the biblical character Job whose faithfulness was tested by an affliction with draining skin sores and pustules. People with this condition have long-term, severe skin infections.

The symptoms are usually present in childhood, but because the disease is so rare, it often takes years before a correct diagnosis is made.

Recent research suggests that the disease is often caused by a genetic change (mutation) -- a change in the STAT3 gene on chromosome 4. How this gene abnormality causes the symptoms of the disease is not well understood. However, people with the disease have higher-than-normal levels of an antibody called IgE.

  • Reviewed last on: 8/1/2011
  • John A. Daller, MD, PhD, Department of Surgery, University of Arkansas for Medical Sciences, Little Rock, Arkansas. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Genetics Home Reference (GHR). Job Syndrome. Feb 2008. Accessed Nov. 13, 2008.

Immune Deficiency Foundation. Immune Deficiency Foundation Patient & Family Handbook. Chapter 12: Hyper IgE Syndrome. 4th ed. 2007. Accessed Nov. 13, 2008.

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