Dupuytren disease

Dupuytren disease/contracture (familial palmar fibromatosis) is a deformity of the hand characterized by the limited mobility of the joints/fingers (usually the ring and small fingers are affected).

Overview

Dupuytren disease or contracture is a deformity of the hand due to progressive fibrosis and shorting of the palmar fascia that leads to the joint contracture and fingers deformity – typically the ring finger and small finger, less frequently the thumb – appear to be fixed in the flexed position and cannot be extended. The disease develops gradually (mostly after the age of 50) and the occurrence of severe hand deformation may be prevented by physical therapy, though it cannot be completely reversed. Nonetheless, the disease may stop progressing and even regress to some extent spontaneously. The presence of Dupuytren contracture may be disabling as it usually affects the right hand and later the left one is involved as well in up to 80% of cases.

Causes

Palmar fascia (aponeurosis) is a triangle composed of connective tissue that lies under the skin in the middle of the palm and extends to the fingers.  In Dupuytren contracture’s this fascia thickens due to fibrosis and eventually leads to palm deformation. However, what triggers the fibrosis remains unknown, though there are some suspicions regarding probable genetic predispositions. Probably the genes controlling the Wnt signaling pathway that regulates the proliferation and differentiation of the cells may be involved in the disease pathogenesis as the fibrosis is the result of abnormal connective tissue distribution.

Risk factors

  • Males are more likely to develop Dupuytren contracture. Moreover, men tend to have worse palmar deformation in comparison to women;
  • Europeans and individuals of European descent also have a higher risk of developing Dupuytren disease;
  • Those who have relatives with Dupuytren contracture are also art risk;

Other risk factors for developing the hand contracture include:

  • Exposure to vibration;
  • Alcohol abuse and liver dysfunction;
  • Smoking;
  • Diabetes mellitus;
  • Hyperlipidemia;
  • Thyroid disorders;
  • History of hand traumatic injury;
  • Some medications administration (for example, anticonvulsants);

Symptoms

Palm deformityProliferative phase during which the fibroblasts undergo proliferation. In such early disease, only small nodules on the palm may be detected. Usually there is no pain, however, a person may experience tenderness and itching in the affected region.

Involutional phase during which the abnormal cord is being formed. Further distribution of the fibrotic tissues results in the occurrence of the cord extending from the palm to the digits.

A residual phase is characterized by the subsequent formation of the contracture and loss of its function.

Diagnosis

The diagnosis can be made by looking at the affected limb. Sometimes ultrasonography may be performed – it will reveal the palmar aponeurosis thickening.

Treatment

There is no definitive treatment for Dupuytren disease.

In mild disease, steroid injections were proven effective as well as physical and occupational therapy.  

To lessen the hand deformity (the contracture is >30-40º) and in order to restore its functionality surgical intervention (fasciotomy and aponeurectomy) can be performed during which the pieces of fibrotic fascia are removed. Other possible treatment options include needling (a needle is used to puncture and ruin the abnormal tissues) and enzyme injections (collagenase Clostridium histolyticum is injected in the palm and breaks down the affected parts of the fascia).

 

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