Otulipenia is the result of the loss-of-function mutation of OTULIN, which encodes a deubiquitinase, causing an increase in canonical NF-κB pathway activation, accumulation of poly-ubiquitinated OTULIN target proteins, e.g., NEMO, RIPK1, ASC, TNFR1, and elevated levels of secreted proinflammatory cytokines, such as IL-6 and TNF (45). The gene discussed is OTULIN; the disease is autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.