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1 OMIM reference -
2 associated genes
6 signs/symptoms
PROTEIN INTERACTIONS: 1
2 OMIM references -
1 associated gene
10 signs/symptoms
Dermatofibrosarcoma protuberans
Fibronectin glomerulopathy

COL1A1 FN1
PDGFB


INTERACTOME
ASSOCIATIONS

(click on a score value to see the evidence)
COL1A1
(0.52)
FN1



Citations in the biomedical literature:


Dermatofibrosarcoma protuberans
COL1A1 PDGFB
Fibronectin glomerulopathy
FN1



Dermatofibrosarcoma protuberans
Fibronectin glomerulopathy

Synonym(s):
- DFSP

Synonym(s):
- GFND
- Glomerulopathy with fibronectin deposits

Classification (Orphanet):
- Rare genetic disease
- Rare oncologic disease
- Rare skin disease
Classification (Orphanet):
- Rare genetic disease
- Rare renal disease

Classification (ICD10):
(no data available)
Classification (ICD10):
- Diseases of the genitourinary system -

Epidemiological data:
Class of prevalence: 1-5 / 10 000
Average age onset: variable
Average age of death: adult
Type of inheritance: sporadic
Epidemiological data:
Class of prevalence: <1 / 1 000 000
Average age onset: variable
Average age of death: -
Type of inheritance: autosomal dominant

External references:
1 OMIM reference -
1 MeSH reference: C538219
External references:
2 OMIM references -
No MeSH references

Dermatofibrosarcoma protuberans
Fibronectin glomerulopathy

Very frequent
- Erythema / erythematous lesions / erythroderma / polymorphous erythema
- Skin / cutaneous neoplasm / tumor / carcinoma / cancer (excluding melanoma)
- Soft tissue sarcoma / cancer / tumor / liposarcoma / myosarcoma
- Subcutaneous nodules / lipomas / tumefaction / swelling
- Thick skin / pachydermia / orange skin

Frequent
- Chronic skin infection / ulcerations / ulcers / cancrum



Very frequent
- Autosomal dominant inheritance
- Chronic arterial hypertension
- Edema of the legs / lower limbs
- Functional anomalies of the kidney and the urinary tract
- Hematuria / microhematuria
- Nephrotic syndrome
- Proteinuria
- Renal failure
- Renal glomerular defect / glomerulopathy

Occasional
- Intracranial / cerebral / meningeal hemorrhage