Author: Hon A/Prof Amanda Oakley, Dermatologist, Waikato Hospital, Hamilton, New Zealand, 2004.
Job syndrome, also called hyper-IgE syndrome, is the name given to a rare inherited immune deficiency characterised by severe infections of the skin and other organs.
Clinical features of Job syndrome include:
The cause of Job syndrome is unknown but it is thought to be a specific genetic abnormality affecting chromosome 4q. The result is a defective immune response involving T lymphocytes, neutrophils and the cytokines they produce, especially interferon-gamma. Excessive levels of interferon-gamma result in marked elevation of immunoglobulin E.
There is no known cure for Job syndrome. Antibiotics are used to control bacterial infections, often requiring a longer course of treatment than is usually necessary. Several doctors from different specialties may need to help care for affected children.
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