What is hyper IgE 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.

What diseases cause high IgE?

Diseases which cause the elevation of serum IgE levels include atopic diseases (asthma, allergic rhinitis, atopic dermatitis, urticaria), parasitic diseases, cutaneous diseases, neoplastic diseases, and immune deficiencies [3].

What immune deficiencies cause high IgE levels?

Clinical Presentation of Hyper IgE Syndrome AR-HIES with DOCK8 deficiency is particularly common in areas of the world with high consanguinity rates (intermarriage among close relatives), where its occurrence may exceed that of AD-HIES.

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.

How is Hyper-IgE syndrome treated?

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.

How is CVID diagnosis?

The diagnosis of CVID is primarily established by testing for low blood (serum) IgG immunoglobulin concentrations ranging from severely reduced (<100 mg/dL) to just below adult normal range (500-1200 mg/dL). In addition, laboratory testing may reveal normal or, in some cases, reduced numbers of circulating B cells.