HIE has variable expressivity and is associated with multiple abnormalities. The most common findings are recurrent skin abscesses pneumonia with pneumatocele development, and high serum levels of IgE. Facial, dental, and skeletal features are also associated with this syndrome.
The pathophysiology of Job syndrome is not completely understood. Patients consistently have a poor, delayed hypersensitivity response to antigens. This delayed response is associated with alterations in T-lymphocyte populations and various interleukin and cytokine abnormalities.