OTULIN and Sepsis: Six carriers were apparently healthy, one-third of patients expressed relatively milder respiratory infections, and the other one-third expressed the severe phenotypes including necrotizing pneumonia, necrotizing cellulitis, recurrent furunculosis, abdominal sepsis, spontaneous abscess, and sepsis shock, thus indicating that rare heterozygous mutations of OTULIN confer predisposition to severe necrosis, typically, but not exclusively, after infection with Staphylococcus aureus.