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What is Hyper IgE syndrome? Hyper IgE syndrome (HIES) is also known as Job's syndrome. It is a primary immunodeficiency where recurrent skin boils, eczema and pneumonia are seen. What are the symptoms of Hyper IgE syndrome? Symptoms of Job's syndrome include eczema, recurrent abscesses, pneumonia and recurrent candida infections. Eczema usually occurs in the creases behind the ears, the back, buttocks and scalp. Boils and abscesses are commonly caused by bacteria called as staphylococcal aureus.
As a consequence of the deficiency in CD40 ligand, the T-lymphocytes in patients with X-linked Hyper IgM (XHIGM) are unable to instruct B-lymphocytes to switch their production of immunoglobulins from IgM to IgG, IgA and IgE.iagnosis of Hyper IgM Syndromes. XHIGM should be considered in any boy presenting with severe recurrent respiratory infections or an opportunistic infection who has low or absent IgG and normal or elevated IgM levels. Failure to express CD40 ligand on activated T-cells is a characteristic finding.