NFKB1 and autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive: Otulipenia, also known as OTULIN-related autoinflammatory syndrome (ORAS), is an autosomal recessive autoinflammatory disease due to mutations in the FAM105B gene, which encodes OTULIN, a Met-1 specific deubiquitinase that acts as a negative regulator of the NF-κB signaling pathway (10).