Among specific IEI with more than 10 patients registered in the KNPIDR, skin manifestations were present in 100% of the patients with dedicator of cytokinesis 8 (DOCK8) deficiency, hyper IgE syndrome (HIGE) [both signal transducer and activator of transcription 3 (STAT3) deficiency and unspecified], and ataxia-telangiectasia (AT) and in 82% of recombination activation gene 1 (RAG1) deficiency, and they were among the presenting signs in 79%, 100%, 83%, and 89% of them, respectively (Table 3). This evidence concerns the gene DOCK8 and hyperinsulinemic hypoglycemia, familial, 4.