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Progression of Dupuytren's
Progression of Dupuytren's Contracture
What Happens As Dupuytren's Contracture Progresses
In most cases the disease does not progress beyond nodules and pitting.
Sometimes, the disease starts to create linear structures under the skin called cords, which typically (but not always) run up the palm from the nodules into the fingers.
The disease then sometimes causes these cords to contract, often forming increasingly sharp ridges in the palm and lower joints of the fingers, and sometimes, as the disease develops further, dragging in the first phalanges of the associated finger (technically, the metacarpophalangeal joint and the proximal interphalangeal joint) and forcing the finger into the characteristic irreversible flexion contracture of the disease.
Many people only go to the doctor about the disease when their finger starts to contract in this way. For many people, in fact, the contracture is the first symptom that they notice.
How Fast Does Dupuytren's Progress?
From an actuarial perspective, most people with Dupuytren's disease die before it develops beyond nodules or cords.
Putting this less dramatically, the chances are that someone who has discovered nodules in their palm will never have to deal with crippled hands.
The activity of the disease varies from person to person, and, for those with bilateral Dupuytren's disease, between hands.
It sometimes progresses steadily, but in most cases it progresses by fits and starts. Sometimes the nodules and cords may even soften and diminish, and in rare cases the disease has spontaneously disappeared.
Most severely deformed hands have taken several years to develop, but cases are known in which a finger has contracted a few weeks after the lumps in the palm were first noticed.
Will My Fingers Contract?
In a large enough population, somewhere between 2% and 7% of people with nodules in their palms will develop cords before the end of the year. A similar percentage who start the year with cords will develop 10 or more degrees of contracture.
Where Can I Get Dupuytren's Disease?
Dupuytren’s disease is almost always confined to the palm and fingers, but can sometimes occur in the wrist or, rarely, in the forearm. Quite commonly, a form of Dupuytren's disease called Garrod pads, or knuckle pads, develops on the outside (the back) of the knuckle joints (technically, the dorsum of the proximal interphalangeal joints). These lumpy pads can sometimes be painful.
Nodules like those of Dupuytren's disease have been reported elsewhere in the body. These nodules, called nodular fasciitis, appear in the superficial fascia - usually in the upper torso and upper arms - and typically afflict people in their 20s and 30s. They sometimes appear very quickly - in a matter of weeks - and normally disappear again.
A few people with Dupuytren's disease develop fibromatosis on the back of the hand.
What Is Happening to my Hand?
In simple terms, the normal process of wound healing and associated scarring is running a little hot. Cells whose job it is to create, maintain and dissolve collagen, the strong elastic tissue that gives muscle and tendons their form, are doing their job a little too well, reproducing themselves a little too enthusiastically and not dying off when they are supposed to.
In slightly greater detail, something in your genes is instructing your body to set up conditions similar to those that prevail when you have wounded yourself. In these conditions the cells called fibroblasts that create and destroy collagen, multiply and at the same time produce more collagen than they destroy.
The fibroblasts are found mainly in the fascia of the palm and sole, a tough sheet of collagen-rich tissue that helps give the palm of the hand and sole of the foot the mechanical structure and strength that these areas need. This excess collagen heaps up as nodules.
Tiny fibres run between the nodules and the skin above them, binding skin to nodule.
At some point the fibroblasts begin to generate long protein molecules that have the capacity to contract. These modified fibroblasts, called myofibroblasts, begin to shorten the collagen by an ingenious ratchet mechanism.
This continuous shortening rucks up the skin above the nodules and cords, while gradually tightening the cords. The ever-tighter cords reel in the finger joints, with the result that the finger begins to curl in the characteristic contracture.
In much greater detail, the histology and biochemistry of the disease turns out to be extremely complex. Less is known about the genetics, but the genetics, too, seems not to be simple.
At What Age Will I Get Dupuytren's Contracture?
Both diseases are considered to be diseases of middle and old age because the onset is typically seen in people in their fifth or sixth decade. But people of any age can get it, and there are even recorded cases of particularly unfortunate people being born with the disease. Younger people are less likely to develop the symptoms, but unfortunately, when younger people do develop the disease, the disease is likely to be more aggressive, and to progress faster. Such people are said to have a strong diathesis for the disease.
Will Dupuytren's recur?
It’s not so much that it recurs as that it never goes away. The genes that set you up for Dupuytren's and Ledderhose disease don’t go away, and the abnormal biochemistry never changes. So after any intervention – particularly a surgical one – all you have done is alleviate a symptom for a while, but if the disease was active before the intervention it has no reason to calm down after the intervention.
What Are The Signs That Dupuytren's Disease Is Active?
The key element is change – change in the appearance and feel of the diseased area over the last few months. Activity in Dupuytren's disease means that nodules are growing and hardening, dimples may be appearing and deepening and cords may be emerging, lengthening or tightening. In Ledderhose disease the lumps may be becoming more distinct or more painful. Particularly with Dupuytren's disease, you may also feel a tingling or tickling that seems to indicate active disease, sometimes together with a hot feeling in the palms. In some people the palms may also feel unusually damp.