autoinflammatory syndrome, familial, Behcet-like 1
MONDO:0800045Mondo
Findings
No curated finding names autoinflammatory syndrome, familial, Behcet-like 1 yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
- Inheritance
- Autosomal dominant inheritance
- Onset and course
- Infantile onset · Juvenile onset · Young adult onset · Childhood onset
HPO, annotations 2026-09-02
Features
14 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Oral ulcerHPOHP:0000155
- 11 of 11 reported patients
- Genital ulcersHPOHP:0003249
- 10 of 11 reported patients
- Polyarticular arthritisHPOHP:0005764
- 5 of 11 reported patients
- Skin rashHPOHP:0000988
- 4 of 11 reported patients
- Anterior uveitisHPOHP:0012122
- 3 of 11 reported patients
- Antinuclear antibody positivityHPOHP:0003493
- 3 of 11 reported patients
- Lupus anticoagulantHPOHP:0025343
- 3 of 11 reported patients
- ColitisHPOHP:0002583
- 2 of 11 reported patients
- Decreased total lymphocyte countHPOHP:0001888
- 2 of 11 reported patients
- Recurrent feverHPOHP:0001954
- 2 of 11 reported patients
- ChoreaHPOHP:0002072
- 1 of 11 reported patients
- Hemolytic anemiaHPOHP:0001878
- 1 of 11 reported patients
Show the remaining 2
- Ileal ulcerHPOHP:0032024
- 1 of 11 reported patients
- ThrombocytopeniaHPOHP:0001873
- 1 of 11 reported patients
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- TNFAIP3HGNC:11896
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2023
- Strong · PanelApp Australia · Autosomal dominant · 2025
- Moderate · Ambry Genetics · Autosomal dominant · 2018
Where it sits
Other names
4 names
Resolves to: autoinflammatory syndrome, familial, Behcet-like 1
- Also called
- Behçet-like disease due to HA20Behçet-like disease due to haploinsufficiency of A20hereditary paediatric Behçet-like diseasehereditary pediatric Behçet-like disease