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Sterile multifocal osteomyelitis with periostitis and pustulosis
1 OMIM reference -
1 associated gene
2 connected diseases
No signs/symptoms info
Disease Type of connection
B-cell chronic lymphocytic leukemia
Glucocorticoid resistance
Synonym(s):
- Autoinflammatory disease due to interleukin-1 receptor antagonist deficiency
- DIRA
- Interleukin-1 receptor antagonist deficiency
- OMPP

Classification (Orphanet):
- Rare genetic disease
- Rare immune disease
- Rare skin disease
- Rare systemic or rheumatologic disease

Classification (ICD10):
(no data available)

Epidemiological data:
Class of prevalence: <1 / 1 000 000
Average age onset: neonatal/infancy
Average age of death: -
Type of inheritance: autosomal recessive
External references:
1 OMIM reference -
No MeSH references

Gene symbol UniProt reference OMIM reference
IL1RN P18510147679
No signs/symptoms info available.