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Dermatofibrosarcoma protuberans

1 OMIM reference -
2 associated genes
6 signs/symptoms

PROTEIN INTERACTIONS: 1
COMMON SIGNS: 1

1 OMIM reference -
1 associated gene
19 signs/symptoms

Dermatofibrosarcoma protuberans

Acrogeria

COL1A1	(UniProt - OMIM)		COL3A1	(UniProt - OMIM)
PDGFB	(UniProt - OMIM)


INTERACTOME ASSOCIATIONS (click on a score value to see the evidence)	PDGFB	(0.68)	COL3A1

Citations in the biomedical literature:

Dermatofibrosarcoma protuberans		Acrogeria
	Synonym(s): - DFSP		Synonym(s): - Acrogeria, Gottron type - Acrometageria - Gottron syndrome

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

	Classification (ICD10): (no data available)		Classification (ICD10): (no data available)

	Epidemiological data: Class of prevalence: 1-5 / 10 000 Average age onset: variable Average age of death: adult Type of inheritance: sporadic		Epidemiological data: (no data available)

	External references: 1 OMIM reference - 1 MeSH reference: C538219		External references: 1 OMIM reference - 1 MeSH reference: C538187


COMMON SIGNS	- Chronic skin infection / ulcerations / ulcers / cancrum

Dermatofibrosarcoma protuberans		Acrogeria
	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		Very frequent - Anomalies of skin, subcutaneous tissue and mucosae - Fine hair - Hyperextensible joints / articular hyperlaxity - Irregular / in bands / reticular skin hyperpigmentation - Lipoatrophy - Premature ageing - Short stature / dwarfism / nanism - Skin hypoplasia / aplasia / atrophy - Thin skin Frequent - Beaked nose - Intellectual deficit / mental / psychomotor retardation / learning disability - Micrognathia / retrognathia / micrognathism / retrognathism - Rippled skin - Scoliosis - Small foot - Small hand / acromicria - Telangiectasiae of the skin Occasional - Dysostosis / chondrodysplasia / osteodysplasia / osteochondrosis / skeletal dysplasia