These include classical autosomal dominant HIES due to STAT3 loss-of-function (LOF) and autosomal recessive entities linked to variants in ZNF341, PGM3, and genes more recently associated with HIES such as SOCS1 genes and CARD11 (Caspase recruitment domain family member 11) (3–7). Here, CARD11 is linked to hyper-IgE syndrome.