高免疫球蛋白E综合征

Hyper- immunoglobulin E syndrome

高免疫球蛋白E综合征（HIES）是一个多系统疾病，以湿疹、皮肤脓肿、皮肤和肺部复发性葡萄球菌感染、肺大疱形成、念珠菌病、嗜酸性粒细胞增多和血清IgE水平升高为特征。辅助性T淋巴细胞17数量减少或缺如，STAT3基因显性负性杂合突变有助于疾病诊断。使用必要的抗生素，是预防化脓性肺炎、防止肺囊腔形成和支气管扩张的必需措施。

Hyper-immunoglobulin syndrome （HIES） is a multisystem disorder characterized by eczema, skin abscesses, recurrent staphylococcal infections of the skin and lungs, pneumatocele formation, candidiasis, eosinophilia, and elevated serum levels of IgE.The number of helper T lymphocyte 17 cells is reduced or absent, and dominant-ne-gative heterozygous mutation in STAT3 gene are helpful for the diagnosis of the disease.The use of prophylactic antibio-tics is a necessary measure to prevent suppurative pneumonia, pulmonary cystic cavity formation and bronchiectasis.