Hyper-IgE syndrome is a rare primary immunodeficiency [30], which consists of three major features, including (1) hyper-elevated serum IgE levels, (2) enhanced susceptibility to any types of infection, and (3) eczema/atopic dermatitis/asthma bronchiale/allergic reaction, which we called the hyper-IgE syndrome symptomatic triad [16]. Here, IGHE is linked to hyper-IgE syndrome.