immune disorders
Hyper IgE Syndrome
Introduction
Hyper-IgE 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 .) IgE is one of 5 types of Antibodies , which help defend the body against infection. The level of IgE is elevated in hyper-IgE syndrome, but this is not the cause of the immunodeficiency. Other parts of the immune system are defective. Why levels of IgE are high is unknown. Hyper-IgE syndrome is a Primary immunodeficiency . It may be inherited in one of two ways: As an Dominant disorders : That is, only one gene for the disorder, one from either parent, is required. As an Recessive disorders : That is, two genes for the disorder, one from each parent, are required. How hyper-IgE is inherited depends on which gene is affected. Both forms cause similar symptoms. 