NFKB1 and autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive: Otulipenia (also known as OTULIN-related autoinflammatory syndrome) is an autosomal recessive autoinflammatory disease caused by mutations in the FAM105B gene coding for OTU deubiquitinase with linear linkage specificity (OTULIN, a Met-1-specific deubiquitinase that downregulates the NF-kB signalling pathway).