What causes hyper IgE syndrome?

What causes 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 happens 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.

What is Hyperimmunoglobulin E syndrome?

(Hyperimmunoglobulinemia E Syndrome; Buckley Syndrome) 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.

What immune deficiencies cause high IgE levels?

Markedly elevated serum IgE levels are observed in 5 primary immune deficiencies (PIDs): hyper-IgE syndrome (HIES), Wiskott-Aldrich syndrome (WAS), immunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX), Omenn syndrome, and atypical complete DiGeorge syndrome.

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 hyper IgE treated?

How is Hyper IgE syndrome treated?

Can Hyper IgE be cured?

Is IgE an autoimmune disease?

There is accumulating evidence to suggest that IgE plays a significant role in autoimmunity. The presence of circulating self-reactive IgE in patients with autoimmune disorders has been long known but, at the same time, largely understudied.