Hyper IgE Syndrome, also known as Job's syndrome, is a rare primary immunodeficiency disorder characterized by recurrent infections, eczema, and elevated levels of immunoglobulin E (IgE) in the blood. It is caused by genetic mutations that affect the immune system's ability to fight off infections.
Due to its rarity, the prevalence of Hyper IgE Syndrome is relatively low. According to available medical literature, it is estimated to affect approximately 1 in every 1 million individuals worldwide. However, it is important to note that prevalence rates may vary across different populations and regions.
Hyper IgE Syndrome can present in different forms, including autosomal dominant and autosomal recessive inheritance patterns. The autosomal dominant form is more common, accounting for the majority of cases. It typically manifests in early childhood, with symptoms such as recurrent skin and lung infections, chronic eczema, and skeletal abnormalities.
While Hyper IgE Syndrome is a rare condition, its impact on affected individuals can be significant. Early diagnosis and appropriate management are crucial in order to minimize complications and improve quality of life for those living with this immunodeficiency disorder.