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- Minegishi Yoshiyuki
- Department of Immune Regulation, Tokyo Medical and Dental University Graduate School Japan Science and Technology Agency (JST), Core Research for Evolution Science and Technology (CREST), Tokyo Medical and Dental University Graduate School
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- Saito Masako
- Department of Immune Regulation, Tokyo Medical and Dental University Graduate School
この論文をさがす
抄録
Hyper-IgE syndrome (HIES) is a primary immunodeficiency disorder characterized by atopic manifestations and susceptibility to infections with extracellular bacteria and fungi. Atopic manifestations include atopic dermatitis-like skin lesion and extremely high serum IgE levels. Most of the extracellular bacterial infections are caused by Staphylococcus aureus, which is associated with milder inflammation compared to normal. Recent studies have revealed that the most cases of the HIES are caused by dominant negative mutations in STAT3 gene. Cutaneous manifestations of HIES includes newborn rash, eczematoid dermatitis, cold abscesses, mucocutaneous candidiasis, and coarse texture of the facial skin. Impaired Th17 cell development due to the defective IL-6 signaling in T cells and impaired induced regulatory T (iTreg) cell generation due to defective IL-10 signaling in dendritic cells may, at least in part, account for the cutaneous pathology of HIES.<br>
収録刊行物
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- Allergology International
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Allergology International 61 (2), 191-196, 2012
一般社団法人日本アレルギー学会