Dupuytren’s
Disease
Definition
Dupuytren’s disease is a condition causing localised
thickening of the fascia of the palm and fingers. The fascia
is a sheet of tissue lying under the palmar skin, but above
the tendons which bend the fingers and above the nerves
and blood vessels going to the fingers. In the fingers themselves
the fascia is less obvious, but can be thought of as layer
of tissue under the skin and in front of the tendons.
Thickening of the fascia causes lumps in the skin (nodules);
pits in the skin and in more severe disease the thickenings
may bend the fingers towards the palm “Dupuytren’s
contracture”.
Incidence/age/sex
About 5% of the Caucasian population have Dupuytren’s
disease. The disease is virtually confined to Caucasian
races and even amongst white populations it is most common
in those populations of Northern Europe or those who are
descended from Northern Europeans (e.g. some Australians).
Males are affected two to three times more commonly than
females. The disease tends to appear with increasing frequency
with increasing age. In Scandinavian populations (highest
incidence) men age 70 - 75 years have an incidence of nearly
40% and in women the disease has its highest frequency between
the ages of 85 - 90 years (25%). About 25% of Anglo-Saxon
males over 60 years of age will have some evidence of Dupuytren’s
disease.
Causes/preventions
There are several conditions that are associated with Dupuytren’s
disease however the exact cause remains unknown. There is
a tendency for Dupuytren’s disease to run in families;
nearly 70% of first degree relatives of an individual with
Dupuytren’s disease will also be affected. Individuals
probably inherit a tendency to develop the disease, which
might or might not appear depending upon the presence or
absence of other factors.
Associations with Dupuytren’s
disease include:
• Epilepsy - the incidence
of Dupuytren’s in epileptics may be as high as 40%.
Some studies have suggested an association with the anti-epilepsy
drug phenobarbitone, rather than the epilepsy itself.
• Alcohol consumption
- several studies have found an association between alcoholism
and Dupuytren’s disease. There is conflicting data
on whether alcoholic liver disease (cirrhosis) is associated
with Dupuytren’s.
• Diabetes - Dupuytren’s
disease is associated with diabetes; however in many patients
the diabetes is not diagnosed. May be as many as 40% of
diabetics will have some degree of Dupuytren’s disease.
In diabetics the disease is often mild and rarely comes
to the attention of a doctor.
• Smoking - one study
has suggested that smokers are nearly twice as likely as
non-smokers to have Dupuytren’s disease.
• Injury - there are
many anecdotal reports of the development of Dupuytren’s
disease following injury of different types; in many cases
this is not possible to prove. Based upon the history of
a single injury to the hand plus evidence of tissue injury
plus the presence of Dupuytren’s disease in the immediate
vicinity one study found that 6% of patients could have
had an injury as an associated factor for their condition.
There is no difference in the incidence of Dupuytren’s
disease between manual and non-manual workers.
Signs & Symptoms
People with Dupuytren’s disease may develop thickening
in the palmar skin; usually this is first noticed as a nodule
in, and under, the skin of the palm. There may be a small
pit in the palmar skin. With progression of the condition
the thickenings may develop into cord like structures which
pass into the fingers. These thickenings may cause contractures
(bending) of the fingers. The disease most commonly affects
the ring and little finger; although any digit may be involved.
The condition may affect both hands and may be associated
with a similar problem in the feet and occasionally with
an upward bend in the erect penis (Peyronie’s disease).
Individuals rarely consult their doctor before the condition
causes bending of the fingers; pain is unusual. The bent
fingers may catch on objects; cause difficulty with placing
the hand flat; may make wearing gloves difficult; may may
facial washing difficult and interfere with putting the
hand into confined spaces, e.g. a pocket.
Complications of disorder
Untreated the disease may cause gradually worsening contractures
of the fingers. The rate and degree at which this occurs
is varied and in some individuals does not happen. If the
finger joints are left in a bent state for a long period
it may never be possible to straighten them, even with appropriate
treatment.
Tests
No special test are needed; a doctor can make the diagnosis
based on the story given by a patient and the findings of
an examination.
Treatment
Surgical
The treatment of Dupuytren’s disease is surgery. However,
surgery is not necessary in all patients affected by Dupuytren’s
disease. Surgery is necessary for patients who are experiencing
functional problems; who have disease which is progressing
quickly and if disease affects the middle joint (proximal
interphalangeal joint) of any finger. If the proximal interphalangeal
joint of any finger is bent by Dupuytren’s disease
early surgery is usually advised, because if left in a bent
position, for prolonged periods, it may be impossible to
straighten at a later date.
Many individuals, with no functional problems and non-progressive
disease, are happy to be reassured that their problem is
not serious and require no treatment other than reassurance.
Surgery for Dupuytren’s disease involves an operation
under either general anaesthetic or regional anaesthetic
(the arm is made “numb”, but the patient stays
awake).
Under anaesthetic cuts are made in the finger and palm to
remove the Dupuytren’s disease (fasciectomy). Sometimes
the cord-like structures are simply cut without removal
of the diseased fascia (fasciotomy). Fasciotomy has a very
high recurrence rate and is usually reserved for particular
situations. After surgery a splint is usually worn continuously
for one week and then at night only for up to 3 months.
After the splint is removed exercises (hand therapy) are
necessary to regain mobility in the hand. Hand therapy may
be needed for several weeks. Any stitches are removed after
2 - 3 weeks.
Postoperative complications after fasciectomy are common
(up to 20%). Sometimes the wounds are slow to heal and sometimes
part of the wounds are left open to heal up by themselves.
The surgery may rarely damage the nerves carrying feeling
from the involved fingers - this may result in a finger
which is numb; this may be temporary or permanent. Rarely
the hand may become unexpectedly stiff and painful after
the surgery; this may be particularly difficult to treat
and require a prolonged period of hand therapy and special
pain management techniques.
Outcome
In patients with longstanding contractures it may not be
possible to fully straighten all of the involved digits;
the proximal interphalangeal joint is particularly troublesome
and if this joint has been affected an average residual
contracture of 30° has been reported.
It is not possible to cure Dupuytren’s disease by
surgery. Many patients have their disease controlled such
that further functional problems do not occur. However,
in a percentage of people the disease will come back in
the affected digit (recurrence of disease) or will develop
and involve other digits (extension of disease). Male patients,
patients with disease in both hands; recurrent disease;
a positive family history; similar disease elsewhere or
early age of onset are more likely to have aggressive disease.
