Hyper-immunoglobulin E syndrome / 中华实用儿科临床杂志

ABSTRACT

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.