Loss-of-function mutations in IL36RN, which encodes IL-36Ra, cause a recessively inherited autoinflammatory keratinization disease known as deficiency of IL-36Ra (DITRA) (IL36RN [MIM: 605507]) [39,40,41,42], because IL-36Ra’s role is to suppress excessive IL-36 signaling. Here, IL36RN is linked to hyperinsulinemic hypoglycemia, familial, 4.