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Hyperimmunoglobulin E syndrome

Definition:

Hyperimmunoglobulin E syndrome is a suspected genetic defect that produces high levels of the antibody immunoglobulin (IgE). It causes serious skin and lung infections as well as eczema .

Alternative Names:

Job syndrome; Hyper IgE syndrome

Causes, incidence, and risk factors:

Hyperimmunoglobulin E syndrome is also know as Job syndrome, after the biblical character Job whose faithfulness was tested by a lifelong affliction of draining skin sores and pustules . People with this condition have chronic and severe skin infections.

It is an extremely rare disease, and the cause is unknown. However, the bacteria Staphylococcus aureus is believe to most commonly trigger the infections related to the disease. Other common triggers include Streptococcus pneumoniae , Haemophilus influenzae , Candida albicans , and the herpes virus.

Hyper IgE syndrome is, in most cases, inherited in a dominant fashion, meaning that it can run in families with high frequency.

References:

Cohen J, Powderly WG. Infectious Diseases . 2nd ed. New York, NY: Elsevier; 2004.

Hoffman R, Benz EJ, Shattil SS, et al. Hematology: Basic Principles and Practice . 4th ed. Orlando, Fl: Churchill Livingstone; 2005.

Review Date: 7/25/2007
Reviewed By: Donald Accetta, MD, MPH, President, Allergy & Asthma Care, PC, Taunton, MA. Review provided by VeriMed Healthcare Network.

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