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Contracture of palmar fascia(DUPC1)

MedGen UID:
41672
Concept ID:
C0013312
Disease or Syndrome
Synonyms: DUPC1; Dupuytren contracture; DUPUYTREN CONTRACTURE 1; Dupuytren's disease of palm, with contracture
SNOMED CT: Contracture of palmar fascia (410813000); Palmar fibromatosis (203045001); Palmar fascial fibromatosis (203045001); Dupuytren disease of palm (203045001); Dupuytren's disease of palm (203045001)
 
HPO: HP:0005679
Monarch Initiative: MONDO:0007476
OMIM®: 126900
Orphanet: ORPHA79142

Definition

Dupuytren contracture is the most common heritable disorder of connective tissue. It is a disease of the soft tissues of the palm and fingers characterized by a progressive thickening and shortening of the fascial structures that normally provide support to the glabrous skin of the palm. Although it can also be a sporadic disorder, the inherited form is most frequently observed among people of Nordic descent. There is a male: female ratio of greater than 3:1 (Hu et al., 2005). Dupuytren contracture has been associated with multiple fibroproliferative conditions, including Peyronie disease (171000), knuckle pads (149100), congenital generalized fibromatosis (228550), juvenile fibromatosis (228600), and frozen shoulder, suggesting a common underlying defect in wound repair (Hu et al., 2005). [from OMIM]

Clinical features

From HPO
Contracture of palmar fascia
MedGen UID:
41672
Concept ID:
C0013312
Disease or Syndrome
Dupuytren contracture is the most common heritable disorder of connective tissue. It is a disease of the soft tissues of the palm and fingers characterized by a progressive thickening and shortening of the fascial structures that normally provide support to the glabrous skin of the palm. Although it can also be a sporadic disorder, the inherited form is most frequently observed among people of Nordic descent. There is a male: female ratio of greater than 3:1 (Hu et al., 2005). Dupuytren contracture has been associated with multiple fibroproliferative conditions, including Peyronie disease (171000), knuckle pads (149100), congenital generalized fibromatosis (228550), juvenile fibromatosis (228600), and frozen shoulder, suggesting a common underlying defect in wound repair (Hu et al., 2005).
Joint contracture of the hand
MedGen UID:
56382
Concept ID:
C0158113
Finding
Contractures of one ore more joints of the hands meaning chronic loss of joint motion due to structural changes in non-bony tissue.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVContracture of palmar fascia
Follow this link to review classifications for Contracture of palmar fascia in Orphanet.

Conditions with this feature

Contracture of palmar fascia
MedGen UID:
41672
Concept ID:
C0013312
Disease or Syndrome
Dupuytren contracture is the most common heritable disorder of connective tissue. It is a disease of the soft tissues of the palm and fingers characterized by a progressive thickening and shortening of the fascial structures that normally provide support to the glabrous skin of the palm. Although it can also be a sporadic disorder, the inherited form is most frequently observed among people of Nordic descent. There is a male: female ratio of greater than 3:1 (Hu et al., 2005). Dupuytren contracture has been associated with multiple fibroproliferative conditions, including Peyronie disease (171000), knuckle pads (149100), congenital generalized fibromatosis (228550), juvenile fibromatosis (228600), and frozen shoulder, suggesting a common underlying defect in wound repair (Hu et al., 2005).
Induratio penis plastica
MedGen UID:
10629
Concept ID:
C0030848
Disease or Syndrome
Fibromatosis arising from the soft tissues of the penis. It is characterized by the presence of spindle-shaped fibroblasts, and an infiltrative growth pattern. It causes the penis to bend when it becomes erect.
Cranioacrofacial syndrome
MedGen UID:
338947
Concept ID:
C1852512
Disease or Syndrome

Professional guidelines

PubMed

Ruettermann M, Hermann RM, Khatib-Chahidi K, Werker PMN
Dtsch Arztebl Int 2021 Nov 19;118(46):781-788. doi: 10.3238/arztebl.m2021.0325. PMID: 34702442Free PMC Article
Rayan GM
J Bone Joint Surg Am 2007 Jan;89(1):189-98. doi: 10.2106/00004623-200701000-00026. PMID: 17256226
Hurst LC, Badalamente MA
Hand Clin 1999 Feb;15(1):97-107, vii. PMID: 10050246

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