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Diseases and Conditions
Dupuytren's contracture
From MayoClinic.com
Special to CNN.com

Introduction

Dupuytren's contracture is a rare hand deformity in which the connective tissue (fascia) under the skin of the palm thickens and scars. Knots (nodes) and cords of tissue form under the skin, often pulling one or more of the fingers into a bent (contracted) position. Though the fingers affected by Dupuytren's contracture bend normally, they can't be straightened, making it difficult to use your hand. Dupuytren's contracture complicates everyday activities such as placing your hands in your pockets, putting on gloves or shaking hands.

Dupuytren's contracture is rarely painful, though sometimes the bumps of tissue on your palm can be sensitive to touch. Men are more likely than women to develop Dupuytren's contracture. It's more common in older adults, usually developing in people in their 50s and 60s. Dupuytren's contracture is more common in whites of Northern European heritage. The condition rarely affects people of color.

Some doctors prefer the term Dupuytren's disease, rather than Dupuytren's contracture, since some people don't experience the bent fingers — the contracture part of the disease.

Signs and symptoms

Dupuytren's contracture usually begins as a thickening of the skin on the palm of your hand. As Dupuytren's contracture progresses, the skin on the palm of your hand may appear dimpled.

A firm lump of tissue may form on your palm. This lump may be sensitive to the touch, but usually isn't painful.

In later stages of Dupuytren's contracture, cords of tissue form under the skin on your palm. Cords may extend up to your fingers. As these cords tighten, your fingers may be pulled toward your palm, sometimes severely. The ring finger and the little finger are most commonly affected, though the middle finger may also be involved. Only rarely are the thumb and index finger affected.

Dupuytren's contracture often affects both hands, though one hand is usually affected more severely than the other.

Dupuytren's contracture usually progresses slowly, over several years. Occasionally it can develop over weeks or months. In some people it progresses steadily and in others it may start and stop. However, Dupuytren's contracture never regresses.

Risk factors

Although the precise cause of Dupuytren's contracture hasn't been identified, a number of factors are believed to increase your risk of the disease, including:

  • Family history. A propensity to develop Dupuytren's contracture may be passed through families, though researchers haven't identified a gene mutation that might identify who's at risk.
  • Alcoholism. It isn't clear whether drinking itself or the liver damage that can result increases the risk of Dupuytren's contracture.
  • Epilepsy. Dupuytren's contracture is more common in people who've had epilepsy for many years. Some doctors believe the risk may come from the anticonvulsant medications that some people with epilepsy take.
  • Diabetes. People with type 1 diabetes or type 2 diabetes have an increased risk of tender tissue lumps on the palms of the hands, but they usually don't develop contracted fingers. It isn't clear if these tissue lumps form because of Dupuytren's contracture or because of some other condition.

When to seek medical advice

Make an appointment with your doctor if you experience hand pain, changes to the skin on your palms, or any other signs and symptoms that concern you. Dupuytren's contracture usually doesn't cause pain and often doesn't interfere with your ability to carry out everyday tasks.

Screening and diagnosis

Your doctor conducts a physical exam to determine whether you have Dupuytren's contracture. Your doctor may conduct an exam called a table top test. In this test, you place your hand palm-down on a table and gently press on the back of your hand. You'll also be asked questions about your signs and symptoms. Your doctor will want to know whether you're having difficulty using your hands; for instance, are you able to extend your fingers to put on gloves or to shake someone's hand.

Your doctor usually doesn't need further tests to determine whether you have Dupuytren's contracture. However, he or she may order other tests to rule out associated conditions, such as diabetes.

Complications

Dupuytren's contracture can make it difficult to perform certain functions using your hand. Since the thumb and index finger aren't usually affected, many people don't experience much inconvenience or disability. But as Dupuytren's contracture progresses, it can limit your ability to fully open your hand.

People with Dupuytren's contracture may experience other connective tissue disorders, including:

  • Plantar fibromatosis. Sometimes called Ledderhose disease, plantar fibromatosis occurs on the sole of the foot and is identical to Dupuytren's contracture. Lumps and cords of tissue form on the sole, and the toes may be pulled down, making it difficult to walk. This rare complication is most common in people with a severe familial form of Dupuytren's contracture called Dupuytren's diathesis.
  • Peyronie's disease. This rare tissue disease affects the penis. Scar tissue forms under the skin of the penis, causing a bend in an erect penis. This complication is rare and may also be a risk in people with Dupuytren's diathesis.
  • Knuckle pads (Garrod's nodes). Pads of tissue may form on the tops of your fingers, over the middle knuckle (proximal interphalangeal joint). Knuckle pads are more common in people with Dupuytren's diathesis.

Treatment

Many people with Dupuytren's contracture never require treatment. Often the disease progresses slowly and has little impact on your ability to use your hands for everyday tasks. If you aren't experiencing pain or disability, you may choose to wait and see if Dupuytren's contracture progresses. Your doctor may ask you to come in for checkups every few years, usually to conduct the table top test and monitor any progression of Dupuytren's contracture in your hands. Or, your doctor may ask you to try the table top test at home on your own and make an appointment if you notice your condition is worsening.

Surgery for Dupuytren's contracture is reserved for people who experience pain and disability from the disease. Doctors recommend this approach because no cure exists for Dupuytren's contracture. While surgery can improve hand function, it doesn't necessarily prevent a recurrence of Dupuytren's contracture. Sometimes the disease returns to the same spot on the hand, other times it reappears in other places on the hand.

Types of surgery
What type of surgery you undergo for Dupuytren's contracture depends on factors such as your age, the degree of contracture in your fingers, and the condition of the skin and bones of your hand. Types of surgical procedures include:

  • Releasing cords of tissue (subcutaneous fasciotomy). Subcutaneous fasciotomy is used in people who can't undergo more extensive surgery or prefer to avoid the risks of an extensive surgery — for instance, older people and people in poor health. In this procedure, a surgeon severs the cords of tissue under the skin. It can be an open procedure, where the surgeon cuts open the skin with a scalpel. Or it can be performed as an outpatient procedure using a needle to gain access to the cords of tissue. Dupuytren's contracture is likely to recur after subcutaneous fasciotomy. Subcutaneous fasciotomy works best when Dupuytren's contracture is limited to the palm of the hand, since the procedure can injure nerves in the fingers.
  • Partial tissue removal (partial fasciectomy). Partial fasciectomy is the most common procedure for Dupuytren's contracture. During partial fasciectomy, the surgeon removes as much of the diseased tissue as possible. Surgeons can't always remove all of the diseased tissue, since it can be difficult to identify tissue in very early stages of the disease. Diseased tissue may also attach to the skin, making it difficult to remove. For this reason, it's common for Dupuytren's contracture to recur after partial fasciectomy, though it's usually less severe and may not require additional surgery.
  • Complete tissue removal (complete fasciectomy) with skin grafting. Younger people and those with the highest chance for recurrence might consider complete fasciectomy to completely remove the tissue on the palm of the hand. The diseased tissue in Dupuytren's contracture usually attaches to the underside of the skin on your palm and fingers, so it may be necessary to remove the skin in order to completely remove the tissue. The removed skin can be replaced with skin from another part of your body. Recurrence is rare after complete fasciectomy. However, this procedure carries the highest risk of complications, including finger stiffness or an inability to bend the fingers. This can be more disabling than the original condition.
  • Finger amputation. Some people experience recurrent Dupuytren's contracture despite multiple surgeries and may consider amputation. Amputation surgery is most commonly used to remove the little finger.

All surgeries carry risks of bleeding and infection. Discuss any concerns with your doctor.

Depending on the extent of your surgery, you may require therapy to help speed your recovery after the procedure. Less-invasive procedures may require four to six weeks of therapy and more invasive surgery could require three to six months of therapy. Therapy usually involves exercises to improve the ability to move your fingers.

Nonsurgical options
A number of nonsurgical options have been proposed for Dupuytren's contracture, including medications and radiation therapy to the hand. Unfortunately none has proved helpful. Cortisone injections may ease the pain of a tender nodule, but usually doesn't affect the progression of Dupuytren's contracture.

Researchers are currently investigating enzyme injections for Dupuytren's contracture. Promising results have shown that enzymes injected under the skin can break down the knots and cords of tissue. Ongoing clinical trials will show whether this can prevent Dupuytren's contracture from recurring. Until then, enzyme injection is considered experimental and is only available at a few medical research facilities.

May 18, 2006

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