高免疫球蛋白E综合征

摘要

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