Hyper-IgE syndrome. Definition

Medical Definition: hyper-IgE syndrome

Primary immunodeficiency characterized by the remarkable increase in serum IgE, which is unable to fix complement and, therefore, to initiate the inflammatory at the site of infection. This phenomenon is responsible for the main clinical manifestation of this disease, recurrent staphylococcal abscesses and other localized infections, especially in the dermis, lungs and joints. Furthermore, the existence of IgG antibodies to IgE complexes, which bind to mononuclear cells, causing the release of cytokines, which induce bone resorption, promoting osteoporosis and bone fractures. Also called Job syndrome.

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