OTULIN and autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive: Homozygous deficiency of OTULIN results in OTULIN-related Autoinflammatory Syndrome (ORAS), which presents in infancy with neutrophlic nodular panniculitis, lipodystrophy and cutaneous vasculitis, as well as recurrent fevers, diarrhoea, and arthritis, and is characterised by prominent acute phase responses and hypergammaglobulinaemia (163–165).