Hyperimmunoglobulin E syndrome is a rare, inherited disease that causes problems with the skin, sinuses, lungs, bones, and teeth.
Job syndrome; Hyper IgE syndrome
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.
Bone and tooth defects, including fractures and losing the baby teeth late
Call your health care provider if you have symptoms of Job syndrome.
There is no proven way to prevent Job syndrome. Good general hygiene is helpful.
Some doctors may recommend preventive antibiotics for people who many infections, especially with Staphylococcus aureus. This treatment does not change the condition, but it can lessen its complications.
Genetics Home Reference (GHR). Job Syndrome. Feb 2008. Accessed Nov. 13, 2008.
Alpen A Patel, MD, Department of Allergy & Immunology, Kaiser Permanente Health Care, Lutherville Timonium, MD. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.