"Hyperimmunoglobulin E (hyper IgE) syndrome (HIES), also known as Job syndrome, consists of a heterogeneous group of complex hereditary combined B- and T-cell immune deficiency diseases characterised by recurrent Staphylococcus aureus chest infections, characteristic coarse facial appearance and dental problems with an eczema-like pruritic dermatitis."
"Hyperimmunoglobulin E (hyper IgE) syndrome (HIES), also known as Job syndrome, consists of a heterogeneous group of complex hereditary combined B- and T-cell immune deficiency diseases characterised by recurrent Staphylococcus aureus chest infections, characteristic coarse facial appearance and dental problems with an eczema-like pruritic dermatitis."
"maybe be associated with DOCK8 deficiency and phosphoglucomutase 3 (PGM3) deficiency"
"In hyper IgE syndrome recurrent chest infections in childhood with Staphylococcus aureus are usually less symptomatic than in immunocompetent children. Hence in most cases, delayed treatment results in severe damage to the lung parenchyma. Besides, even after adequate treatment of the acute infection, the healing process causes severe parenchymal injury, bronchiectasis, scarring, and large pneumatocele formation."
"After several pulmonary infections, the lungs become more susceptible to secondary indolent infections with Pseudomonas and Aspergillus which are difficult to treat, with usual worsening of the parenchymal and bronchial damage, resulting in a vicious circle of lung infections and injury."
"There are at least two types of hyper IgE syndrome 2:"