Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED; autoimmune polyendocrine syndrome type 1; OMIM 240300) is caused by mutations in the autoimmune regulator gene (AIRE) that lead to failure of negative selection of autoreactive T cells and to impaired function of regulatory T cells.1 Hypoparathyroidism and primary adrenal insufficiency are the most common early endocrinopathies, but new autoimmune manifestations may appear throughout life. This evidence concerns the gene AIRE and primary adrenal insufficiency.