Hyper IgE immunodeficiency is a congenital T –cell immunodeficiency characterized by facial changes, recurrent abscesses, retained teeth, skin changes and elevated immunoglobulin E. The disease manifestations are largely attributed to the body’s inability to mount an appropriate immune response due to poor INF-gamma production and subsequent loss of its chemotactic properties.
Hyper IgE syndrome is also called Job's syndrome.
T cells lack the ability to signal other immune cells resulting in abnormal chemotaxis due to a deficiency in IFN-gamma production.
T cells lack the ability to signal other immune cells due to a deficiency in IFN gamma production. In addition to promoting TH1 differentiation, IFN-gamma promotes adhesion and binding required for leukocyte migration.
Leonine facies is a descriptive term used for a deeply furrowed ‘lumpy' face with prominent supercilliary arches that is seen in Hyper IgE syndrome.
Without the appropriate immune response, the body is unable to respond to staph infections in the usual way. Instead, the reaction is truncated and described as cold. Patients have several non-inflamed boils on the skin.
Patients fail to lose baby teeth resulting in two sets of teeth.
Also known as atopic dermatitis, this is a rash characterized by intense pruritus often presenting on the flexor surfaces of the skin as erythematous papules.
Patients with Hyper IgE syndrome often have characteristic red hair.
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