These human hyper-IgE syndromes (HIES) include the autosomal dominant Job ́s syndrome and autosomal recessive PGM3 (phosphoglucomutase 3) and SPINK5 (Serine Peptidase Inhibitor Kazal Type 5) syndromes, that can be successfully treated with anti-IgE antibody therapy [85] or stem cell transplantation [86]. Here, SPINK5 is linked to hyper-IgE recurrent infection syndrome 1, autosomal dominant.