Hyper-IgE Syndrome (HIES); Job´s Syndrome
Hyper-IgE syndrome is an inherited multisystem disease characterized by the classical triad of abscesses, pneumonia, and extremely elevated serum IgE.
Immunological and Non-Immunological Clinical Features
Job´s syndrome was first reported 1966 by Davis et al. on the basis of the case reports of two young girls with eczema, cold abscesses of the skin, and pneumonias (Davis et al. 1966). It was named Job´s syndrome in reference to the biblical Job whose body was affected with boils. Based on inheritance, an autosomal dominant (AD-HIES) and an autosomal recessive (AR-HIES) form of hyperimmunoglobulin E syndrome have been reported.