Haploinsufficiency of A20 (HA20), associated with mutations in the TNFAIP3 gene, is an early-onset autoinflammatory syndrome often mimicking the clinical spectrum of Behçet's disease (BD), including recurrent oral and genital ulcers, polyarthritis, skin involvement, and gastrointestinal manifestations (29). Here, TNFAIP3 is linked to autoinflammatory syndrome.