Severe combined immunodeficiency (SCID), some combined immunodeficiencies (CIDs) (e.g., autosomal recessive (AR) Hyperimmunoglobulin E syndrome (HIES), Nuclear factor-kB essential modulator (NEMO) deficiency) and PIDs with defective Th17 immunity (e.g., autosomal dominant (AD) HIES, Caspase recruitment domain-containing protein 9 (CARD9) deficiency and AR Autoimmune polyendocrinopathy–candidiasis–ectodermal dystrophy (APECED) are examples of inborn errors of immunity associated with chronic mucocutaneous candidiasis (CMCC) (Table 3) [125,133]. The gene discussed is IKBKG; the disease is hyper-IgE syndrome.