<unk>  (DIRA) is an autosomal recessive, genetic autoinflammatory syndrome resulting from mutations in IL1RN, the gene encoding the<unk>  1<unk><unk> . The mutations result in an abnormal protein that is not secreted, exposing the cells to unopposed<unk>  1 activity. This results in sterile multifocal osteomyelitis, periostitis (inflammation of the membrane surrounding the bones), and pustulosis due to skin inflammation from birth.
Deficiency of the interleukin-1–receptor antagonist
Leukocyte adhesion molecule deficiency
Terminal complement pathway deficiency
Neutrophil-specific granule deficiency
Acquired C1 esterase inhibitor deficiency
Mendelian susceptibility to mycobacterial disease
CD25 deficiency
Immunodeficiency with hyperimmunoglobulin M