Alternatively, vasculitis can also be a leading feature especially in the recently defined monogenic AID (Otulipenia, Deficiency of Adenosine Deaminase 2-DADA2, Haploinsufficiency of A20) and interferonopathies (STING-associated vasculopathy with onset in infancy-SAVI). Here, TNFAIP3 is linked to autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.