Dupuytren’s Contracture

Dupuytren's Contracture
Dupuytren's Contracture

Overview Of Dupuytren’s Contracture

Dupuytren’s Contracture is a painless thickening and tightening (contracture) of tissue beneath the skin on the palm of the hand and fingers. Dupuytren’s contracture is characterized by a deformity of the hand in which the joints of one or more fingers cannot be fully straightened (extended); their mobility is limited to a range of bent (flexed) positions. The condition is a disorder of connective tissue, which supports the body’s muscles, joints, organs, and skin and provides strength and flexibility to structures throughout the body. In particular, Dupuytren’s contracture results from shortening and thickening of connective tissues in the hand, including fat and bands of fibrous tissue called fascia; the skin is also involved.

In men, Dupuytren’s contracture most often occurs after age 50. In women, it tends to appear later and be less severe. However, Dupuytren’s contracture can occur at any time of life, including childhood. The disorder can make it more difficult or impossible for affected individuals to perform manual tasks such as preparing food, writing, or playing musical instruments.

Dupuytren’s contracture often first occurs in only one hand, affecting the right hand twice as often as the left. About 80 percent of affected individuals eventually develop features of the condition in both hands.

Dupuytren’s contracture typically first appears as one or more small hard nodules that can be seen and felt under the skin of the palm. In some affected individuals the nodules remain the only sign of the disorder, and occasionally even go away without treatment, but in most cases the condition gradually gets worse. Over months or years, tight bands of tissue called cords to develop. These cords gradually draw the affected fingers downward so that they curl toward the palm. As the condition worsens, it becomes difficult or impossible to extend the affected fingers. The fourth (ring) finger is most often involved, followed by the fifth (little), third (middle), and second (index) fingers. Occasionally the thumb is involved. The condition is also known as Dupuytren disease, and “Dupuytren contracture” most accurately refers to later stages when finger mobility is affected; however, the term is also commonly used as a general name for the condition.

About one-quarter of people with Dupuytren’s contracture experience uncomfortable inflammation or sensations of tenderness, burning or itching in the affected hand. They may also feel pressure or tension, especially when attempting to straighten affected joints.

People with Dupuytren’s contracture are at increased risk of developing other disorders in which similar connective tissue abnormalities affect other parts of the body. These include Garrod pads, which are nodules that develop on the knuckles; Ledderhose disease, also called plantar fibromatosis, which affects the feet; scar tissue in the shoulder that causes pain and stiffness (adhesive capsulitis or frozen shoulder); and, in males, Peyronie disease, which causes abnormal curvature of the penis.

Commonly Associated With

Palmar fascial fibromatosis – Dupuytren; Flexion contracture – Dupuytren; Needle aponeurotomy – Dupuytren; Needle release – Dupuytren; Percutaneous needle fasciotomy – Dupuytren; Fasciotomy- Dupuytren; Enzyme injection – Dupuytren; Collagenase injection – Dupuytren; Fasciotomy – enzymatic – Dupuytren; Dupuytren’s Disease

Causes Of Dupuytren’s Contracture

While the cause of Dupuytren’s contracture is unknown, changes in one or more genes are thought to affect the risk of developing this disorder. Some of the genes associated with the disorder are involved in a biological process called the Wnt signaling pathway. This pathway promotes the growth and division (proliferation) of cells and is involved in determining the specialized functions a cell will have (differentiation).

Abnormal proliferation and differentiation of connective tissue cells called fibroblasts are important in the development of Dupuytren’s contracture. The fascia of people with this disorder has an excess of myofibroblasts, which are a type of fibroblast containing protein strands called myofibrils. Myofibrils normally form the basic unit of muscle fibers, allowing them to contract. The increased number of myofibroblasts in this disorder causes abnormal contraction of the fascia and produces excess amounts of a connective tissue protein called type III collagen. The combination of abnormal contraction and excess type III collagen likely results in the changes in connective tissue that occurs in Dupuytren’s contracture. However, it is unknown how changes in genes that affect the Wnt signaling pathway are related to these abnormalities and how they contribute to the risk of developing this disorder.

Other risk factors for developing Dupuytren’s contracture may include smoking; extreme alcohol use; liver disease; diabetes; high cholesterol; thyroid problems; certain medications, such as those used to treat epilepsy (anticonvulsants); and previous injury to the hand.

Symptoms Of Dupuytren’s Contracture

One or both hands may be affected. The ring finger is affected most often, followed by the little, middle, and index fingers.

A small, nodule or lump develops in the tissue below the skin on the palm side of the hand. Over time, it thickens into a cord-like band. Usually, there is no pain. In rare cases, the tendons or joints become inflamed and painful. Other possible symptoms are itching, pressure, burning, or tension.

As time passes, it becomes difficult to extend or straighten the fingers. In severe cases, straightening them is impossible.

Exams & Tests

The health care provider will examine your hands. Diagnosis can usually be made from the typical signs of the condition. Other tests are rarely needed.

Treatment Of Dupuytren’s Contracture

If the condition is not severe, your provider may recommend exercises, warm water baths, stretching, or splints.

Your provider may recommend treatment that involves injecting medicine or a substance into the scarred or fibrous tissue:

  • Corticosteroid medicine relieves inflammation and pain. It also works by not allowing the thickening of the tissue to get worse. In some cases, it heals the tissue completely. Several treatments are usually needed.
  • Collagenase is a substance known as an enzyme. It is injected into the thickened tissue to break it down. This treatment has been shown to be just as effective as surgery.

Surgery may be done to remove the affected tissue. Surgery is usually recommended in severe cases ofDupuytren’s contracture when the finger can no longer be extended. Physical therapy exercises after surgery help the hand recover normal movement.

A procedure called aponeurotomy may be recommended. This involves inserting a small needle into the affected area to divide and cut the thickened bands of tissue. There is usually little pain afterward. Healing is faster than surgery.

Radiation is another treatment option. It is used for mild cases of contracture, when the tissue is not so thick. Radiation therapy may stop or slow down the thickening of the tissue. It is usually done only one time.

Talk to your provider about the risks and benefits of the different kinds of treatments.

Other

Outlook (Prognosis)

Dupuytren’s contracture progresses at an unpredictable rate. Surgery can usually restore normal movement to the fingers. The disease can recur within 10 years after surgery in up to one-half of cases.

Possible Complications

Worsening of the contracture may result in deformity and loss of function of the hand.

There is a risk of injury to blood vessels and nerves during surgery or aponeurotomy.

When to Contact a Medical Professional

Call your provider if you have symptoms of this disorder.

Also, call if you lose feeling in your finger or if your fingertips feel cold and turn blue.

Prevention

Awareness of risk factors may allow early detection and treatment.