The study by Ahonen et al. examines the clinical manifestations and course of autoimmune polyendocrinopathy–candidiasis–ectodermal dystrophy (APECED) in 68 patients from 54 families, ranging in age from 10 months to 53 years. The clinical manifestations varied widely, with 63% of patients having three to five disease components. Oral candidiasis was the most common initial manifestation, followed by hypoparathyroidism and adrenal failure. The earliest endocrine component appeared between 19 months and 35 years of age, and new components continued to develop until at least the fifth decade. The majority of patients had multiple components of the disease, and the first component often appeared in childhood. The study highlights the broad clinical spectrum of APECED and emphasizes the need for lifelong follow-up to detect new components of the disease. The authors also note that the presence of certain HLA-DQ alleles, such as HLA-DQw1.2, is protective against APECED, while HLA-DQw8 increases the risk.The study by Ahonen et al. examines the clinical manifestations and course of autoimmune polyendocrinopathy–candidiasis–ectodermal dystrophy (APECED) in 68 patients from 54 families, ranging in age from 10 months to 53 years. The clinical manifestations varied widely, with 63% of patients having three to five disease components. Oral candidiasis was the most common initial manifestation, followed by hypoparathyroidism and adrenal failure. The earliest endocrine component appeared between 19 months and 35 years of age, and new components continued to develop until at least the fifth decade. The majority of patients had multiple components of the disease, and the first component often appeared in childhood. The study highlights the broad clinical spectrum of APECED and emphasizes the need for lifelong follow-up to detect new components of the disease. The authors also note that the presence of certain HLA-DQ alleles, such as HLA-DQw1.2, is protective against APECED, while HLA-DQw8 increases the risk.