Autoimmune polyglandular syndrome Type 1 (APS1), namely Autoimmune–Polyendocrinopathy-Candidiasis-Ectodermal dystrophy syndrome (APECED, OMIM #240300, ORPHA 3453), is determined by loss-of-function mutations in the autoimmune regulator (AIRE) gene; the presence of at least two of the following disorders—chronic mucocutaneous candidiasis (CMC), chronic hypoparathyroidism (HP) and primary adrenal insufficiency (Addison’s disease, AD)—confirms clinical diagnosis [4]. This evidence concerns the gene AIRE and autoimmune polyendocrine syndrome type 1.