Indeed, two patients from our cohort displayed at onset an IPEX-like phenotype, associating atopic dermatitis, eosinophilia, high serum IgE and severe diarrhea, histologically defined as eosinophilic colitis, but keeping normal number of Tregs and lacking endocrine autoimmunity. This evidence concerns the gene IGHE and immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome.