ODCs

Click node...

1 OMIM reference -
1 associated gene
18 signs/symptoms

PROTEIN INTERACTIONS: 1

1 OMIM reference -
1 associated gene
3 signs/symptoms

Pyogenic arthritis - pyoderma gangrenosum - acne

X-linked severe congenital neutropenia


INTERACTOME ASSOCIATIONS (click on a score value to see the evidence)	PSTPIP1	(0.97)	WAS

Citations in the biomedical literature:

Pyogenic arthritis - pyoderma gangrenosum - acne		X-linked severe congenital neutropenia
	Synonym(s): - FRA - Familial recurrent arthritis - PAPA syndrome		Synonym(s): (no synonyms)

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

	Classification (ICD10): (no data available)		Classification (ICD10): - Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism -

	Epidemiological data: Class of prevalence: <1 / 1 000 000 Average age onset: childhood Average age of death: - Type of inheritance: autosomal dominant		Epidemiological data: Class of prevalence: <1 / 1 000 000 Average age onset: neonatal/infancy Average age of death: - Type of inheritance: x-linked recessive

	External references: 1 OMIM reference - No MeSH references		External references: 1 OMIM reference - No MeSH references

Pyogenic arthritis - pyoderma gangrenosum - acne		X-linked severe congenital neutropenia
	Very frequent - Acne / acnea - Arthritis / synovitis / synovial proliferation - Asthenia / fatigue / weakness - Autosomal dominant inheritance - Chronic skin infection / ulcerations / ulcers / cancrum - Fever / chilling - Pustula / pustulosis - Restricted joint mobility / joint stiffness / ankylosis Frequent - Articular / joint pain / arthralgia - Lymphadenopathy / polyadenopathies - Monoclonal immunoglobulins / gammapathy / dysglobulinemia Occasional - Biological inflammatory syndrome / increased erythrocyte sedimentation rate / CRP - Bone marrow failure / pancytopenia - Functional colopathy / irritable bowel syndrome - Furuncle / cutaneous abscess / hidrosadenitis suppurativa - Insulin-dependent / type 1 diabetes - Myositis - Proteinuria		Very frequent - Agammaglobulinemia / hypogammaglobulinemia / B-cell deficiency - Polynuclear cells / neutrophils anomalies / neutropenia - X-linked recessive inheritance