Hyper-IgE syndrome (HIES) is an inborn error of immunity (IEI) characterized by recurrent infections (e.g., staphylococcal skin abscesses, bacterial pneumonia, chronic mucocutaneous candidiasis), severe atopy, connective tissue abnormalities (e.g., osteopenia, spontaneous fractures, primary tooth retention, joint hyperextensibility), high serum immunoglobulin E (IgE) concentration, eosinophilia, and an impaired inflammatory response [1–5]. Here, IGHE is linked to hyper-IgE syndrome.