Behçet-like syndrome (BLS) is defined as the presence of Behçet syndrome features, or Behçet disease (BD), occurring in association with a distinct clinical and/or pathological defined entity, such as various inborn errors of immunity (IEI), including autoinflammatory conditions related to dysregulation of the nuclear factor kappa B (NF-κB) pathway (e.g., TNFAIP3, NFKB1, RELA, IKK gamma mutations) or DADA2, CSF2, and LIG4 mutations [1]. This evidence concerns the gene TNFAIP3 and Behcet disease.