OTULIN is a deubiquitinase that removes methionine 1 (M1)‐linked polyubiquitin chains conjugated by the linear ubiquitin chain assembly complex (LUBAC), and mutations OTULIN cause OTULIN‐related autoinflammatory syndrome (ORAS), a TNF‐driven autoinflammatory disease in humans. Here, OTULIN is linked to autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.