Baxter et al. previously reported destructive germline variants in the SKIC3 gene in 48 cases (4.17%) of FOXP3-negative IPEX-like disease, suggesting that similar variants may exhibit regional variability in disease prevalence (Baxter et al., 2022). Here, FOXP3 is linked to immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome.