Hall of Fame quarterback John Elway was recently seen on NBC's "Today" show to he has been battling for 15 years -- Dupuytren's contracture. When the former Denver Bronco QB -- now the team's general manager -- was first diagnosed, both his ring fingers were constricted. He was unable even to properly hold a football, Elway said on the show.
"When I saw my hand doctor, the only option was surgery," he said. "I wasn't interested in more surgeries. I've had so many surgeries during my playing career. At that point in time, I didn't want to have another one."
As the disease progressed, and newer treatment options became available, Elway underwent a procedure called enzymatic injection (details below), which now allows him to move his fingers. He is also a spokesperson for a website called to spread awareness of the disease and its treatment options.
What is Dupuytren's Contracture?
Dupuytren's contracture is characterized by a deformity of the hand in which the joints of one or more fingers cannot be fully straightened. Mobility is limited to a range of bent positions. The condition is a disorder of connective tissue. Dupuytren's contracture results from the shortening and thickening of connective tissues in the hand, including fat and bands of fibrous tissue called fascia. The overlying skin can also be involved.
Dupuytren's contracture occurs in about 5% of people in the U.S. The condition is 3 to 10 times more common in people of European descent than in other groups. Sometimes the condition is called "Vikings' Disease."
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% 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 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.
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's 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.
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 proliferation of cells and is involved in determining the specialized functions a cell will have (differentiation).
Abnormal proliferation and differentiation of 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 cause abnormal contraction of the fascia and produce 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.
Treatment of Dupuytren's Contracture
Treatment of Dupuytren's involves breaking apart the cords that are causing the contracture. This can be done in one of three ways: needling, enzyme injections, or surgery.
- Needling: Also called needle aponeurotomy or percutaneous needle fasciotomy. After a local anesthesia is injected, the physician inserts a needle into the thickened tissue to break it apart enough so that the fingers can be straightened. The advantages of this procedure are that it is less invasive, low risk, has a shorter recovery time, and costs less. However, there is a relatively high rate of re-contracture, occurring in up to 80% of patients. The procedure can be repeated if contracture returns.
- Enzyme injections: As the tissue that causes the contractures are rich in collagen, injection of collagenase (produced by Clostridium histolyticum), sold under the trade name Xiaflex, has been shown to weaken the cords of Dupuytren's contracture. Twenty-four hours after the injection, a physician will manipulate the fingers to break up the tightened tissue and restore finger mobility. Although considered a safe procedure, complications can occur, including pain, injury to the overlying skin, swelling, or hematoma in the skin underneath the injection site and, occasionally, tendon rupture. Enzyme injection is more expensive than needling (over $3,000 per vial) but less expensive than surgery.
- Surgical repair: Until recently, surgery was the only treatment option, and is still the treatment of choice for severe contractures. Disadvantages include being more invasive, more painful, and involving a longer rehabilitation period compared to the other two methods. However, surgery provides longer-lasting relief and a longer time until recurrence of symptoms.
Michele R. Berman, MD, and Mark S. Boguski, MD, PhD, are a wife and husband team of physicians who have trained and taught at some of the top medical schools in the country, including Harvard, Johns Hopkins, and Washington University in St. Louis. Their mission is both a journalistic and educational one: to report on common diseases affecting uncommon people and summarize the evidence-based medicine behind the headlines.