Major Histocompatibility Complex (MHC) class II (MHCII) deficiency (MHCII-D), also known as Bare Lymphocyte Syndrome (BLS), is a rare autosomal recessive combined immunodeficiency (CID) due to mutations in genes regulating expression of MHCII molecules [OMIM#209920] (CIITA, RFXANK, RFX5, RFXAP) (1–4). This evidence concerns the gene CIITA and MHC class II deficiency.