Dupuytren’s Contracture and Plantar Fibromatosis

Dr. Greene, my 14-year-old daughter Jaymie has been diagnosed with a connective tissue disorder. The disease has two names — Dupuytren’s contracture in the hands, and Plantar fibromatosis in her feet. We have been told that before she graduates from high school she will be in a wheelchair for the rest of her life. The disease is a genetic type of disease and it comes from my side of the family. They have told us that there is nothing they can or will do for her because she is so young. She has, however, been accepted to a wonderful hospital in Portland, Oregon (Shriner’s Hospital for Crippled Children). What do you know about this disorder, and is there a cure for it? What is the prognosis for her life? Is there any good news that you can give me about a possible future? If not, what can we expect for her? The progress of the symptoms are getting worse fast and she has had some major difficulties lately.
Robi Nelson – Concerned Mom – Portland, Oregon

Dupuytren's Contracture and Plantar Fibromatosis

Dr. Greene’s Answer:

Robi, most parents hold the deep, unquestioned belief that over the next several years we will watch our children grow ever more independent and more mobile as they walk out of our homes into the world beyond. This poignant, long departure that begins at the moment of birth provides a bittersweet backdrop to the many wonderful moments when our children are young.

How jarring, how deeply wrong, it must feel for you to be told that your teenage daughter is going to become progressively less mobile, will soon be wheelchair bound for life, and — at the time she is becoming an adult woman — needs to be cared for at a hospital for crippled children! You are in a discouraging and bewildering situation. Let me tell you what I know of her condition. I’m sorry that you both have to face this, but there is hope with the treatment options now available.

In the palms of our hands and the soles of our feet, we each have a tough fibrous layer called fascia (the palmar fascia and plantar fascia, respectively). In Dupuytren’s contracture (pronounced du-pwe-trahns), one or both of these fibrous layers begins to grow awry. In palmar fibromatosis (“classic” Dupuytren’s contracture) the palmar fascia slowly begins to thicken, and then shorten. The fingers are relentlessly drawn inward into a rigid, misbegotten fist. As flexibility slips away, so does the useful functioning of the hand. In plantar fibromatosis, this same relentless shortening happens in the soles of the feet, drawing the toes downward, folding the feet into a frozen fist, and making it impossible to walk. The foot version is much less common. Either way, untreated Dupuytren’s contracture can be a crippling disease.

Dupuytren’s contracture was first described by Baron Guillaume Dupuytren, a celebrated French surgeon of the early 1800’s who was apparently successful with the surgical treatment of this condition. By carefully cutting the involved fascia he was able to achieve good results — for a while. Whatever had caused the fascia to grow incorrectly before, caused the regrowing fascia to eventually shorten and thicken as well. Thus, for over 100 years the condition was thought to be relentlessly progressive. We now know that it can follow many courses, from quite mild to very severe. There are also more effective treatment options than ever before, and there is real reason to have hope for Jaymie.

Dupuytren’s contracture is a genetic condition that is passed as a dominant trait with “variable penetrance.” This means that, if it runs on your side of the family, it is present in someone in every generation, although it may be so mild as to go unnoticed. Either you or her father must have it, Robi, and one of your parents must have it as well — although neither of you may ever have any symptoms from it at all. Whoever carries this gene will pass it along to about half of his or her offspring.

The real problem in Dupuytren’s contracture is with the DNA in the cells of the fascia. DNA provides the blueprint that instructs the fascia how to grow. Fascial cells with abnormal DNA will eventually produce abnormal fibrous tissue time and time again. Really, then, Dupuytren’s contracture may be classified as a benign tumor of the fascia. I expect that gene therapy will be developed during Jaymie’s lifetime that will correct the root problem and make treatment effective and permanent.

Already, today’s surgical techniques provide better treatment than ever before. By means of electron microscopy and DNA analysis, physicians can differentiate between normal fascia and normal-appearing fascia that will one day cause trouble. By carefully removing all of the involved plantar fascia with a wide margin of normal fascia, surgeons at Brown University have had excellent results in treating plantar fibromatosis (Plastic and Reconstructive Surgery, Feb 1989). Since 1989, other surgical techniques have been developed with positive results. If and when Jaymie undergoes surgery, I would insist on a team with experience treating her condition.

The continuous elongation technique, pioneered by Doctors Messina and Messina of Turin, Italy, is a more recent development in the treatment of Dupuytren’s contracture that appears to further enhance both short- and long-term results (Plastic and Reconstructive Surgery, Jul 1993). Here, a device is affixed to a bone in the hand or foot to provide a steady, painless stretching of the contracting fascia. This preparatory step is used in severe Dupuytren’s contracture before excision of the affected fascia (Journal of Hand Surgery, Jun 1996).

A fortuitous observation gave rise to another line of therapy for Dupuytren’s contracture: the contracture tends not to recur beneath a skin graft! (This suggests to me that the DNA of the overlying skin may be involved somehow in the condition). In those patients with a strong inherited tendency to the production of Dupuytren’s contracture, recurrence may occur or even be anticipated, and the placement of a skin graft strategically at a flexion crease is shown to act as a ‘firebreak’ between areas of potential flare-up of recurrent Dupuytren’s disease. These skin grafts (also called ‘Firebreak’ grafts) are now in use as a means of controlling recurrent Dupuytren’s disease (Australia and New Zealand Journal of Surgery, Jun 1984).

The nonsurgical treatments, radiotherapy and injections of superoxide dismutase, have now been shown not to work. But high potency topical steroid ointments may be of some benefit (Lancet, Aug 1993). On the theory that the DNA damage may be the result of an auto-immune problem (i.e. her body’s immune system is attacking her own fascia), clobetasol ointment has been massaged into the skin above contractures, apparently halting, or at least stalling, the progression of the disease. Recent studies using collagenase injections have been promising (Journal of Hand Surgery, July-Aug 2007).

Dupuytren’s contracture is usually thought of as a disease of the elderly. The condition is very rare in children. In fact, the report on largest grouping of these children that I am familiar with appeared in the Journal of Hand Surgery (February 1996). It described only 9 children (all of whom, by the way, were treated with surgery by age 14).

With most of the questions I receive, I have at least some firsthand knowledge of the condition, either from medical school, residency, or pediatric practice. I have never met a child with Dupuytren’s contracture, and my knowledge is limited to what I have read and learned in lectures.

I was able to find in the National Library of Medicine database 199 articles on Dupuytren’s contractures. I reviewed all of these for which abstracts (in English) were available, and have passed along the highlights to you. You can find citations for all of the articles at: http://www.nlm.nih.gov/databases/index.html

It is my hope that physicians with direct knowledge of this condition will share their experience with us, and I will be sure to pass along to you anything I receive.

I also hope that this information at least gets you started on the path out of your bewildering situation. The future is not at all clear. The images of your child, wheelchair bound for life, may well not be real. This will likely be a long battle, but a combination of contracture release surgery, continuous stretching, firebreak grafts, and perhaps some topical medicines, holds some real promise. And over the next decade, genetic treatment of the underlying problem may powerfully and effectively correct Jaymie’s underlying condition.

All the best,

Note: We received a helpful letter from a reader here at drgreene.com who wished to pass on some information about a new minimally invasive surgery for Dupuytren’s Contracture called “needle aponevrotomy” or “needle fasciotomy”. This is not a pediatric surgery, however if you are interested you can learn more about the surgery at www.dupuytrenscenter.com.

Dr. Alan Greene

Dr. Greene is the founder of DrGreene.com (cited by the AMA as “the pioneer physician Web site”), a practicing pediatrician, father of four, & author of Raising Baby Green & Feeding Baby Green. He appears frequently in the media including such venues as the The New York Times, the TODAY Show, Good Morning America, & the Dr. Oz Show.

  1. Becky Mitchell

    Thank you Dr Greene for the wonderful information about Dupuytens contractor. The thing that frustrates me the most is that much information out there says that it is not painful. That is just not true for everyone.

    I’m 53 and I started getting lumps in my hands around 40. I knew what it was because my grandfather, father, and brother had it. My brothers was the worst. He had surgery which helped for less than 24 hrs. He eventually had his pinky amputated, after getting his hand stuck in a saw. I know my brother’s was painful.

    Another reader mentioned itching. That’s the first discomfort I experienced. But now, I get pain when using a hammer or pliers. My grip strength is very weak. Mine has advanced 4 fold in just a year. Should I expect it to continue to advance further at this rate? And secondly do you think that the skin graft would help me at this point?

  2. Darla Ortiz

    What about immune modulaters?

  3. Denise Brown

    I have Dupuytren’s and Ledderhose. I find the Coffee Talks at DDSG, Dupuytren’s Disease Support Group, a closed Facebook group so helpful. They have had Doctors host these talks where you can ask the doctor a question and get a reply. The members are like family with tons of experience ranging from dormant and on hold, radiation therapy, NA, Xiaflex, surgery, and amputation. The goal of the group is advocacy and global awareness so we can push for more treatment options and maybe a cure one day.

  4. Dave Reifsnyder

    Started in my hands about 20 years ago – both ring fingers, but I got it to subside by doing an exercise where I simply opened my whole hand as wide as I could about fifty times a day. I still do this and I have visible swelling and I guess scarring beneath my skin, but no pain and pretty much full function as long as I keep up with the stretching exercise. I also have it in my feet, which has been more of a problem – lots of pain and swelling comes and goes. The treadmill seems to helpp keep the swelling and pain down. Tried Verapamil ointment for one year on my foot nodules but it did little to nothing. I also feel like I’m getting effects in my ankles and lower legs, but that doesn’t seem consistent with the descriptions of the condition.

  5. Norm Halverson

    left ring and pinky about 10%, pain at base of ring,started about 5 .years ago (I am 73, scandanavian, do not know if father, or mother had it). some stiffness begining in right digits (all). have begun dmso and e protocol. will add serrapeptase when I find right product. and am designing a night time glove with builtin splints.. will keep the forum updated monthly.

  6. Keith Allardice

    I have had the disease in my right hand for about 7 years. It’s fairly advanced with my ring finger & pinkie about 30 deg down with the hand outstretched. The only discomfort is itchiness and stiffness in Winter. My elder brother has it & my father & grandfather had it. All untreated. My grandfather was born in Scotland. My condition does not affect my golf ( I think!!)
    Keith Allardice, Cape Town, SA.

  7. Bonnie Overby

    I am 51 yrs old and both my hands are affected with Dupytren’s as was my father and several of my siblings. I recently learned this can affect the feet and I am sure I also have this in my feet. I also have a neuropathy on the inside of my lower legs starting in my ankles and I am wondering if plantar fibromatosis could be responsible for this neuropathy?

  8. Elizabeth Campbell

    Regarding radiotherapy, many people with Dupuytren’s and Ledderhose are finding that this is the best possible treatment. Do please consider joining the Facebook group ‘DART’ (Dupuytren’s Advocate’s for Radio Therapy). The group consists of lay people (patients) and doctors.

  9. Jose Vazquez

    Dr.Greene, I’m a ,50 yld. Disable veteran, I have two lumps I’m my right hand and three on my left hand at the palm both,no pain at all,also I have one lump on each foot this one cause me a lot of pain more at right foot than left,also seems like still growing, right is bigger than left and the pain some time won’t leave sleep some time. What can I do to get some relieve? I’m desperate! Thanks for any advice provided.

  10. james moriarty

    I am a mailman, 50 yrs old, and have had the lump in the middle of my right hand for a couple of years. No pain, no contracture of the hand, and am an avid golfer. After a round today, noticed 2 similar lumps in the bottom of left foot, also with no pain. Any advice?


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