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Hyper IgE Syndrome

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As a senior expert in the field of IgE research, Creative Biolabs is willing to provide our customers with a wealth of Hyper IgE Syndrome (HIES) -related background information.

Introduction of HIES

HIES is recognized as a complex immunodeficiency disease accompanied by repeated lung and skin infections with bacteria, atopic dermatitis, and extremely high serum IgE levels. The initial high IgE syndrome is characterized by a weakened inflammatory response, accompanied by Staphylococcus aureus skin abscess and pneumonia, followed by emphysema. Clinically, the skin manifestations of patients with HIES are similar to common atopic dermatitis. These immunological findings are usually related to bone and connective tissue abnormalities. Most patients manifest beyond immune system abnormalities, including bone and connective tissue abnormalities, such as scoliosis, osteoporosis, joint hyperextension, unique facial appearance, and deciduous teeth retention.

Clinical features in AD-HIES. Fig.1 Clinical features in AD-HIES. (Yong, 2012)

Genetics

Studies have identified that most autosomal dominant HIES (AD-HIES) are closely related to the mutation of STAT3. AD-HIES patients will also exhibit abnormalities in connective tissue and bones. The multi-system abnormalities in AD-HIES patients confirm that STAT3 not only plays an important role in the immune system, but also has extensive connections with teeth, musculoskeletal, and vascular systems.

Diagnosis

Initially, the HIES scoring system for genetic linkage studies was developed. The system uses scores as the standard to determine the probability of disease. Moreover, children with eczema and significantly elevated IgE levels are usually diagnosed on the basis of atopic dermatitis. With the discovery of STAT3 mutations and evidence of Th17 cell reduction in this group of patients, the diagnosis may be helpful. However, it is very difficult to detect Th17 levels, and it may be easier to detect mutations in the STAT3 gene.

Treatment

Treatment of infections and skin care are the main tasks of HIES management. HIES dermatitis is mainly caused by infection. Therefore, skin treatment includes a bleaching bath and antibiotics. After the use of antibiotics, skin abscesses will be reduced, and sometimes they may be treated in conjunction with surgery. Fungal infection is also a concern. Oral antifungal drugs are usually effective in controlling fungal diseases. There is also a small amount of data showing that some patients have improved after receiving high-dose immunoglobulin therapy. At present, testing the vaccination response and considering treatment for specific populations seems to be a strategy worth considering. Bone marrow transplantation was also utilized in AD-HIES, but its exact effect is not very clear, and it may be accompanied by some adverse reactions.

In recent years, the diagnosis and treatment technology of HIES has continued to advance, and more and more scientists are committed to HIES-related research. If you have any questions about the project, please contact us in time.

Reference

  1. Yong, P. F.; et al. An update on the hyper-IgE syndromes. Arthritis research & therapy. 2012, 14(6): 1-10.

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