Patients affected with autosomal recessive hyper-IgE syndrome (AR-HIES) due to DOCK8 (dedicator of cytokinesis 8) [56] or Tyk2 (tyrosine kinase 2) gene mutation [57] share common features of HIES, such as elevated serum IgE concentration and eosinophilia as well as predisposition to staphylococcal and candidal infections however, different infection profile and clinical features suggest a distinct disease entity. This evidence concerns the gene TYK2 and hyper-IgE syndrome.