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.