In 2016, A20 haploinsufficiency (HA20), caused by heterozygous mutation of the TNFAIP3 gene, was reported to cause an early-onset autoinflammatory disease presenting with BD-like features such as recurrent aphthous stomatitis, genital ulcers, and gastrointestinal symptoms (3). Here, TNFAIP3 is linked to Behcet disease.