ODCs

Cytoscape Web

Click node...

Limited cutaneous systemic sclerosis

6 associated genes
19 signs/symptoms

PROTEIN INTERACTIONS: 1
COMMON SIGNS: 1

1 OMIM reference -
1 associated gene
37 signs/symptoms

Limited cutaneous systemic sclerosis

TRAPS syndrome

CAV1	(UniProt - OMIM)		TNFRSF1A	(UniProt - OMIM)
CCR6	(UniProt - OMIM)
CTGF	(UniProt - OMIM)
HLA-DRB1	(UniProt - OMIM)
IRF5	(UniProt - OMIM)
KIAA0319L	(UniProt - OMIM)


INTERACTOME ASSOCIATIONS (click on a score value to see the evidence)	CAV1	(0.49)	TNFRSF1A

Citations in the biomedical literature:

Limited cutaneous systemic sclerosis		TRAPS syndrome
	Synonym(s): - Limited cutaneous systemic scleroderma		Synonym(s): - Autosomal dominant periodic fever - Familial hibernian fever - TNF receptor 1 associated periodic syndrome - Tumor necrosis factor receptor 1 associated periodic syndrome

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

	Classification (ICD10): - Diseases of the musculoskeletal system and connective tissue -		Classification (ICD10): - Endocrine, nutritional and metabolic diseases -

	Epidemiological data: Class of prevalence: 1-9 / 100 000 Average age onset: adulthood Average age of death: adult Type of inheritance: sporadic		Epidemiological data: Class of prevalence: unknown Average age onset: childhood Average age of death: - Type of inheritance: autosomal dominant

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


COMMON SIGNS	- Nausea / vomiting / regurgitation / merycism / hyperemesis

Limited cutaneous systemic sclerosis		TRAPS syndrome
	Very frequent - Abnormal pigmentary skin changes / skin pigmentation anomalies - Acrocyanosis / Raynaud's phenomenon / vasomotor disorders - Anomalies of skin, subcutaneous tissue and mucosae - Autoimmunity / autoimmune reaction / autoantibodies - Dermal / subcutaneous infiltration / induration - Dry / squaly skin / exfoliation - Irregular / patchy skin hypopigmentation Frequent - Chronic skin infection / ulcerations / ulcers / cancrum - Feeding disorder / dysphagia / swallowing / sucking disorder / esophageal dyskinesia - Gastroesophageal reflux / pyrosis / esophagitis / hiatal hernia / gastroparesia - Periarticular tissue anomaly / extraarticular calcifications - Telangiectasiae of mucosae - Telangiectasiae of the skin Occasional - Claw hand / retracted fingers - Claw toes / retracted toes - Lung fibrosis - Musculo-tendinous retractions - Pulmonary hypertension		Very frequent - Acute abdominal pain / colic - Biological inflammatory syndrome / increased erythrocyte sedimentation rate / CRP - Cutaneous rash - Erysipela - Fever / chilling - Macules - Malabsorption / chronic diarrhea / steatorrhea - Myalgia / muscular pain - Pericardium anomalies / pericarditis / absence / cysts / pericardial effusion Frequent - Abnormal pleura / hydrothorax / pleuresia / pleural effusion / chylothorax - Arthritis / synovitis / synovial proliferation - Constipation - Erythema / erythematous lesions / erythroderma / polymorphous erythema - Hyperleukocytosis / leukocytosis - Intestinal obstruction / ileus - Lymphadenopathy / polyadenopathies - Splenomegaly - Testes inflammation / orchitis Occasional - Articular / joint pain / arthralgia - Cellulitis / panniculitis / pseudocellulitis / inflammation of subcutaneous tissue - Chronic uveitis / blepharitis / episcleritis / scleritis / conjonctivitis / keratitis - Cranial nerves palsy - Dizziness - Ecchymoses - Facial pain / cephalalgia / migraine - Fasciitis - Follicular / erythematous / edematous papules / milium - Myocardium anomalies / myocarditis - Myositis - Paresthesia / dysesthesia / hypoesthesia / anesthesia / numbness - Peritonitis / peritoneal abscess - Pharyngitis - Psychic / behavioural troubles - Sacroiliac joints anomalies - Thoracic / chest pain - Vascularitis / vasculitides / arteritis