Whereas the failure to activate NF-κB quickly and appropriately during microbial challenge resulted in increased susceptibility to infections, the reduced ability to cleave HOIL1 and reduce linear ubiquitination is consistent with unrelenting NF-κB signalling leading to a destructive chronic inflammation of her skin and gastrointestinal tract that was only partially controllable with systemic corticosteroid therapy. The gene discussed is NFKB1; the disease is inflammation.