Hyper-ige syndrome

[Julene]

Hyper-IgE (hyperimmunoglobulinemia E) syndrome is a hereditary immunodeficiency disorder characterized by recurring boils, sinus and lung infections, and a severe rash that appear during infancy. Levels of immunoglobulin E (IgE) are very high.

In infants with hyper-IgE syndrome, abscesses form in the skin, joints, lungs, or other organs.
Blood tests can confirm the diagnosis.
Treatment involves giving antibiotics to prevent or treat infections, creams or drugs to relieve the rash, and drugs that modify the immune system.

(See also Overview of Immunodeficiency Disorders.)
Hyper-IgE syndrome is a primary immunodeficiency disorder. It may be inherited in one of two ways:

As an autosomal (not sex-linked) dominant disorder: That is, only one gene for the disorder, one from either parent, is required.
As an autosomal recessive disorder: That is, two genes for the disorder, one from each parent, are required.

How hyper-IgE is inherited depends on which gene is affected. Why levels of IgE are high is unknown. Both forms cause similar symptoms.