Rare Disease Spotlight: Dupuytren’s Contracture
/By Barby Ingle, PNN Columnist
This month’s rare disease spotlight looks at a painful hand deformity called Dupuytren’s contracture. My father had this condition and I know two other men who have it; one white and one Hispanic. It is most common in older men of Northern European descent, which is why it is known as the “Viking disease.” It occurs in about 5 percent of people in the United States.
Dupuytren’s contracture usually affects the ring and “pinky” fingers, making it difficult to completely straighten them or pick up objects. It develops over time when knots of tissue form under the skin, creating a think cord that can pull one or more fingers into a bended position. Only rarely are the thumb and index finger affected. Dupuytren's can occur in both hands, though one hand is usually affected more severely.
Doctors don’t know what causes Dupuytren's and there is no evidence that hand injuries or occupations that involve the hands contribute to it. My father used to say his condition developed from playing football and getting his fingers smashed between the pads of other players. Interestingly, former NFL quarterback John Elway has Dupuytren’s and recently began appearing in TV commercials promoting a non-surgical treatment.
Dupuytren’s can be very frustrating for those trying to do everyday actives like putting your hands in your pockets, putting on a glove or just shaking hands. As it progresses, the skin on your palm might appear puckered or dimpled. A firm lump of tissue can form on your palm that is sensitive to touch.
I had similar symptoms for several years, but for a very different reason. My right hand and foot curled up due to Reflex Sympathetic Dystrophy (RSD). My symptoms improved after I began infusion therapy in 2009, but my fingers remain affected and the lump in my palm is still sensitive.
My dad’s fingers were so bent up, he had surgery to straighten them. They just curled up again and never functioned well, before or after surgery. This is known as treatment-resistant Dupuytren’s. There is no way to know if you have a treatment resistant version until surgery is tried.
Dupuytren's contracture occurs most commonly in men over the age of 50. Men are also more likely to have severe symptoms than women. The condition tends to run in families so there may be a genetic component that has yet to be confirmed. If you are the first in your family to get it, it’s possible that tobacco and alcohol use may contribute to it developing. My dad had diabetes II, which is also a risk factor, although he had Dupuytren's prior to being diabetic.
For those with mild forms of this condition, you can do a few things to help slow its progress. Start with avoiding projects with tight grip requirements, like mechanical work with small tools, or use padding on your hands to protect them.
In some cases, Dupuytren's does not progress or does so slowly. But if treatment is needed, it may involve surgically removing or breaking apart the cords that are pulling your fingers toward your palm. The choice of procedure depends on the severity of your symptoms and other health problems you may have.
There is a procedure less invasive than surgery where a needle is inserted through your skin. If contractures recur, the procedure can be repeated. This option requires little to no physical therapy, but can damage nerves or tendons in your hand.
There is also a medication, called Collagenase Clostridium Histolyticum, which can be injected into your hand so the provider can try to break up the cord and straighten your fingers. The injections are not widely offered and have similar drawbacks to the needling option.
My father chose surgery because his condition was advanced and he had limited use of his hand. His provider surgically removed the tissue in his palm that was affected by the disease and closed it up with a skin graft. The results were mixed. For a few years, my father could open his hand and spread some of his fingers, but he did not get total strength or movement back and it progressively worsened as he aged. He also had physical therapy while recovering from the surgery, which took about 6 months for him. He said it was worth the try.
If you are looking for a Dupuytren's resource center and support group, one of the better ones I found is run by University of California, San Francisco. They provide a variety of services, including social workers and condition-specific support groups, as well as classes to help patients and their families.
Barby Ingle lives with reflex sympathetic dystrophy (RSD), migralepsy and endometriosis. Barby is a chronic pain educator, patient advocate, and president of the International Pain Foundation. She is also a motivational speaker and best-selling author on pain topics. More information about Barby can be found at her website.
