Considering that anti-TNF therapy is highly effective in ORAS cases (Damgaard et al., 2016, 2019; Zhou et al., 2016), including the current patient, we speculate that enhanced cell death due to decreased OTULIN activity plays a crucial role in the pathogenesis of ORAS. Here, TNF is linked to autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.