Dupuytren’s Contracture

Dupuytren's contracture (also known as "Morbus Dupuytren," "Dupuytren's disease," or "Palmar fibromatosis", and sometimes misspelled as Dupuytren's constricture) is a fixed flexion contracture of the hand where the fingers bend towards the palm and cannot be fully extended (straightened). It is named after Baron Guillaume Dupuytren, the surgeon who described an operation to correct the affliction.

Dupuytren's contracture is caused by underlying contractures of the palmar fascia. The ring finger and little finger are the fingers most commonly affected. The middle finger may be affected in advanced cases, but the index finger and the thumb are nearly always spared. Dupuytren's contracture progresses slowly and is usually painless. In patients with this condition, the tissues under the skin on the palm of the hand thicken and shorten so that the tendons connected to the fingers cannot move freely. The palmar aponeurosis becomes hyperplastic and undergoes contracture.

Incidence increases after the age of 40; at this age men are affected more often than women. After the age of 80 the distribution is about even.

Symptoms

In Dupuytren's disease, the tough connective tissue within one's hand becomes abnormally thick which can cause the fingers to curl and can result in impaired function of the fingers, especially the small and ring fingers. It usually has a gradual onset, often beginning as a tender lump in the palm. Over time, pain associated with the condition tends to go away, but tough bands of tissue may develop. These bands, which are the source of the reduced mobility commonly associated with the condition, are visible on the surface of the palm and may appear similar to a small callus. It commonly develops in both hands and has no connection to dominant or non-dominant hands, nor any correlation with right- or left-handedness.

The contracture sets in slowly, especially in women. However, when present in both hands and when there is associated foot involvement, it tends to progress more rapidly.

Risk factors and possible causes

Dupuytren's disease is a very specific affliction, and primarily affects:

• People of Scandinavian or Northern European ancestry; it has been called the "Viking disease", though it is also widespread in some Mediterranean countries (e.g. Spain and Bosnia) and in Japan.
• Men rather than women (men are ten times as likely to develop the condition)
• People over the age of 40
• People with a family history (60 to 70% of those afflicted have a genetic predisposition to Dupuytren's contracture)
• People with liver cirrhosis

Some suspected, but unproven causes of Dupuytren's contracture include trauma, diabetes, alcoholism, epilepsy therapy with phenytoin and liver disease. There is no proven evidence that hand injuries or specific occupational exposures lead to a higher risk of developing Dupuytren’s disease although there is some speculation that Dupuytren's may be caused or at least the onset may be triggered by physical trauma, such as manual labor or other over-exertion of the hands. However, the fact that Dupuytren's is not connected with handedness casts some doubt on this claim.

Treatment

Dupuytren's contracture is not a dangerous condition, and often no treatment is necessary. Even when treatment is used, there is no permanent way to stop or cure the affliction.

Surgical management consists of opening the skin over the affected cords and excising (removing) the fibrous tissue. The fingers may then be brought out to length with the help of postoperative therapy. The procedure is not curative in that remaining non-affected fascia may still develop Dupuytren's disease later on, and therefore the patient may need repeat surgery. In addition, the thickened fascia often is near to or wrapped around the digital nerves and arteries, so there is some risk of nerve and/or arterial injury.

Needle aponeurotomy

Needle aponeurotomy is a minimally invasive technique where the cords are weakened through the insertion and manipulation of a small needle. Once weakened, the offending cords may be snapped by simply pulling the finger(s) straight. The advantage claimed for needle aponeurotomy is the very small amount of surgery required and the very rapid return to normal activities, but the nodules are not removed and might start growing again.

Collagenase

Currently in phase III of United States Food and Drug Administration (FDA) approval is another promising therapy, the injection of collagenase. This procedure is similar to needle aponeurotomy, but the cords are weakened through the injection of small amounts of an enzyme that dissolves them.

Radiation therapy

Radiation therapy, treatment of Dupuytren's contracture with low energy x-rays, has also shown some promise in trials lacking a control group.

Notable sufferers

Samuel Beckett, Ronald Reagan, Margaret Thatcher, Bill Nighy, David McCallum and David Pinsky are among the most famous patients.