Hyper IgE syndromes(HIES)comprise a group of rare primary immunodeficiency disorders characterized by a triad of atopic dermatitis, recurrent skin and lung infections along with elevated IgE levels.According to clinical and molecular characteristics, HIES can be classified into 2 types: autosomal dominant HIES(AD-HIES)and autosomal recessive HIES(AR-HIES). At present, STAT3, DOCK8, IL6ST and ZNF341 are included in OMIM database.AD-HIES, caused by STAT3 mutations, can present multiple systemic clinical symptoms and even lead to the development of pulmonary structural abnormalities including pneumatoceles and bronchiectasis.AR-HIES, caused by DOCK8, IL6ST or ZNF341 mutations, often present with severe fungal, bacterial or viral infections.Neurologic manifestations are frequent complications, such as vasculitis, hemangioma and cerebral infarction.The mortality rate is high.There is no specific index for the clinical diagnosis of HIES, and the final diagnosis still needs to be combined with genetic diagnosis.This article reviews the above genes and their possible pathogenesis.