Over the past few years, the use of dupilumab in IEI has gained recognition, with increasing reports of its effective and safe treatment for conditions such as CARD11-associated atopy with predominant NF-κB signaling interference (CADINS) [33, 34], hyper-IgE syndrome induced by dominant negative variants in STAT3 [35–39], as well as DOCK8 deficiency [40],WAS [41] and other IEIs [42, 43]. Here, DOCK8 is linked to hyperinsulinemic hypoglycemia, familial, 4.