APECED is diagnosed in patients who have 2 of the triad of adrenal insufficiency (Addison disease), hypoparathyroidism, and chronic mucocutaneous candidiasis. Some allelic variants, in particular the IranianJewish polyglandular syndrome, are recognized with only parathyroid involvement. Polyendocrinopathy can include IDDM, hypergonadotropic hypogonadism, and autoimmune thyroid disease. Other autoimmune manifestations can include hepatitis, malabsorption, alopecia, vitiligo, and pernicious anemia. Typically candidiasis appears in early childhood, followed by hypoparathyroidism and then Addison disease, but presentation and severity can vary.