Académique Documents
Professionnel Documents
Culture Documents
Defination:
This is a condition characterised by a flexion deformity of one or
more fingers due to a thickening (nodular hypertrophy) and
shortening of the palmar aponeurosis (superficial palmar fascia)
Epidemiology
About 60 per cent of patients give a family history.
The condition is inherited as an autosomal dominant trait
It is more common in males than females;
the prevalence increases with age,
Onset at an early stage usually means aggressive disease.
There is a high incidence in epileptics receiving phenytoin
therapy;
associations with
Diabetes,
Smoking,
Alcoholic cirrhosis,
AIDS and
Pulmonary tuberculosis
There is a contentious and weak association with injury
to the hand.
Pathology
The essential problem in Dupuytren’s disease is proliferation of
myofibroblasts;
After an initial proliferative phase, fibrous tissue within the
palmar fascia and fascial bands within the fingers contracts,
Causing flexion deformities of the MCP and PIP joints.
Fibrous attachments to the skin lead to puckering.
The digital nerve is displaced or enveloped, but not invaded, by
fibrous tissue. Occasionally the plantar aponeurosis also is
affected.
Clinical feature:
The patient – usually a middle-aged man
Complains of a nodular thickening in the palm.
Gradually this extends distally to involve the ring or little finger.
Pain may occur early on but is seldom a marked feature.
Often both hands are involved, one more than the other.
The palm is puckered, nodular and thick.
If the subcutaneous cords extend into the fingers they may
produce flexion deformities at the MCP and PIP joints.
Sometimes the dorsal knuckle pads are thickened (Garrod’s
pads).
Treatment –
no surgery is needed unless the contracture progresses to functional
impairment.
Surgical
A. Operation is indicated
If the deformity is a nuisance or rapidly progressing.
(In particular, PIP joint contractures can become irreversible.)
B. The aim is
Reasonable, not complete correction.
Surgery does not cure the disease, it only partially corrects the
deformity
Correction of is
myofibroblast the MCP
the joint is cell
dominant more predictable
type than contracture.
in Dupuytren the PIP
Myo + Fibroblast
This is a contractile cell with increased type 3 collagen, possibly
originating from a transformed perivascular smooth muscle cell.
The Dupuytren fibroblast also has higher levels of a smooth muscle
actin and contractility than a normal fibroblast. Most investigators now
agree that in Dupuytren contracture the subcutaneous nodules and
cords are formed by fibroplasia and by hypertrophy of already existing
fibers of the palmar fascia. Millesi theorized that the pathological tissue
arises only through changes in the existing fibers of the palmar fascia
and not by the formation of new tissue. Luck suggested that the
subcutaneous nodules develop first, and cords form proximally in
response to intermittent stress resulting in contractures. According to
this concept,
the nodules progress through proliferative, involutional, and residual
stages.
In the proliferative stage, the nodules are young, non–stress aligned
fibroblasts that expand and displace the subcutaneous tissues and fuse
to the skin. The nodules typically appear around the distal palmar
crease over the metacarpophalangeal joint and distally over the
proximal interphalangeal joint, but never over the distal
interphalangeal joint.