What is hyper-IgE syndrome?
What is hyper-IgE syndrome?
The hyper IgE syndromes (HIES) are rare primary immune deficiencies characterized by elevated serum IgE, rash and recurrent bacterial infections of the skin and lung.
Who discovered Job’s syndrome?
David et al. first described reported “Job syndrome” in 1966 in two patients with eczema, recurrent pulmonary infections, and cold lung abscesses.
Is hyper-IgE syndrome curable?
Treatment of hyper-IgE syndrome consists of lifelong prophylactic antistaphylococcal antibiotics (usually trimethoprim/sulfamethoxazole). Dermatitis is treated with skin hydration, emollient creams, antihistamines. and, if infections are suspected, antibiotics.
Which antibodies are elevated in hyper-IgE syndrome?
Hyper IgE Syndrome (HIES) is a rare primary immunodeficiency disease characterized by eczema, recurrent staphylococcal skin abscesses, recurrent lung infections, eosinophilia (a high number of eosinophils in the blood) and high serum levels of IgE.
Is Hyper IgE syndrome rare?
Autosomal recessive hyper IgE syndrome (AR-HIES) is a rare primary immunodeficiency disorder.
Why is it called Job’s syndrome?
Hyperimmunoglobulin E syndrome is also called Job syndrome. It is named after the biblical character Job, whose faithfulness was tested by an affliction with draining skin sores and pustules. People with this condition have long-term, severe skin infections.
Why is there Hyper IgE in Job syndrome?
What is Hyper IgE Syndrome (Job’s Syndrome)? Hyper IgE syndrome is an immune deficiency caused by a genetic mutation in either STAT3 (autosomal dominant, also known as Job’s syndrome) or DOCK8 (autosomal recessive). There may be other mutations in other genes that are not known at this time.
How many people have Hyper-IgE syndrome?
AD-HIES is rare, affecting fewer than 1 per million people worldwide.
Is Hyper-IgE syndrome genetic?
Hyper IgE syndrome is an immune deficiency caused by a genetic mutation in either STAT3 (autosomal dominant, also known as Job’s syndrome) or DOCK8 (autosomal recessive). There may be other mutations in other genes that are not known at this time.
How do you treat Job syndrome?
No definitive therapy is available for the treatment of hyper-IgE syndrome (HIE syndrome or Job syndrome). The mainstay of treatment is the control of bacterial infections. Early incision and drainage followed by the intravenous administration of antibiotics are used for cutaneous infections.
What age does Job syndrome present?
Age. In a recent chart review, the average age of diagnosis was 11.5 years. However, the diagnosis can be made in infancy or adulthood.
Why is it called Job syndrome?