Section 16. Immune Disorders

Chapter 184. Immunodeficiency Disorders

Topics: Introduction | X-Linked Agammaglobulinemia | Selective Antibody Deficiency | Common Variable Immunodeficiency | Transient Hypogammaglobulinemia of Infancy | Chronic Mucocutaneous Candidiasis | DiGeorge Anomaly | Ataxia-Telangiectasia | Severe Combined Immunodeficiency Disease | Wiskott-Aldrich Syndrome | Hyperimmunoglobulinemia E Syndrome | Chronic Granulomatous Disease | Immunodeficiency due to Spleen Disorders

Hyperimmunoglobulinemia E Syndrome

Hyperimmunoglobulinemia E syndrome (hyper-IgE syndrome, or Job-Buckley syndrome) is a hereditary immunodeficiency disorder with very high levels of IgE and normal levels of other antibody classes, resulting in recurring infections.

In most people with hyperimmunoglobulinemia E syndrome, neutrophils--a type of white blood cell that is also a phagocyte--are abnormal. (Phagocytes are cells that ingest and kill bacteria.) The cause is unknown. The skin, joints, lungs, or other organs may be infected, usually with Staphylococcus bacteria. Many people with this disorder have weak bones and therefore many fractures. Some people have symptoms of allergy, such as eczema, nasal stuffiness, and asthma. Facial features may be coarse.

Antibiotics, often trimethoprim-sulfamethoxazole, are given continuously or intermittently for the staphylococcal infections. Lifespan depends on the severity of the lung infections.