Dupuytren's syndrome: definition, symptoms, causes, diagnosis and treatment

By Cristiano Antonino On Jun 23, 2023

A chronic and progressive disease, Dupuytren’s syndrome (or Dupuytren’s disease) affects the palmar band of the hand: one or more fingers flex permanently and irreducibly, causing joint stiffness and making simple gestures difficult

It is named after Baron Guillaume Dupuytren who presented his findings in Paris in 1831

However, even today the syndrome has unknown causes, although it is believed that it may be due to local hypoxia; furthermore, it is known for certain that there is a certain genetic predisposition, in fact the incidence is increased in those with a positive family history.

However, there are treatments that can slow down its progression and improve hand function, but there are still many unresolved issues.

Dupuytren’s syndrome mostly affects men, especially those in their fifth or sixth decade, and almost always of Caucasian race (it is nicknamed ‘Viking disease’, as it is particularly prevalent in Northern Europe).

In this population, the prevalence is around 10%. Usually bilateral, it affects the ring and little finger in 70-80% of cases.

Dupuytren’s syndrome: what is it?

Dupuytren’s syndrome causes permanent contracture of one or more fingers of the hand, often accompanied by the occurrence of nodules in several places in the palm.

The palm of the hand, beneath the skin surface, has what is known as the palmar aponeurosis (palmar fascia): this is a strong fibrous membrane, and can be regarded as a dependency of the tendon of the long palmar muscle and the transverse ligament of the carpus.

It is made up of bundles of connective tissue with a longitudinal course joined by transverse bundles.

Lying under the skin, it lines the main muscles of the hand and the blood vessels that pass through it.

The onset is typically in old age with a small, hard nodule just under the skin at palm level.

It may then worsen, leading to contracture in flexion of the fingers of the hand with inability to extend (typically fourth finger, followed by fifth and third fingers).

It is typically not painful, but may cause mild pain and itching. It is a tumour of benign origin and completely harmless.

Dupuytren’s syndrome: the causes

Dupuytren’s syndrome arises when the connective tissue of the palmar fascia thickens, and the tendons of the finger or fingers closest to it shorten.

It is unclear, to date, what causes the thickening with the consequent formation of the nodule and the shortening of the tendons (which in turn causes the flexion): the most accepted theory is a genetic one.

In fact, sufferers often have parents or siblings affected by the same disease.

Local ischaemic factors are also thought to be involved in the pathogenesis.

According to the most recent research, there are certain risk factors

diabetes
alcohol intake
smoking
use of anticonvulsants (drugs used to treat epilepsy)
old trauma to the wrist

Dupuytren’s syndrome: symptoms

The primary symptom of Dupuytren’s syndrome is the appearance of a painful lump in the palm of the hand, usually in the middle or ring finger area and up to a centimetre in diameter. Initially, the pain may be annoying, but as time passes, it fades.

At the same time, the fingers begin to bend until they contract permanently.

The symptoms are thus

palmar nodule
digital contractures in bending
pain in the palm and hand (never as a predominant symptom)
skin sclerosis (connective thickening at the level of the dermis and hypodermis, which manifests itself as hardening of the affected skin)
itching
difficulty gripping objects

The patient suffering from Dupuytren’s syndrome also notices it visually, as well as by touch.

However, by the time the symptomatology is evident, months (or even years) have passed since the onset of the disease.

At an advanced stage, the patient is no longer able to straighten flexed fingers and therefore experiences great difficulty in performing everyday gestures, from holding cutlery to driving. It is therefore essential to consult one’s doctor in good time.

Dupuytren’s syndrome: the diagnosis

The diagnosis of Dupuytren’s syndrome consists of a simple objective test: the general practitioner, by analysing the symptomatological manifestations, can verify the actual existence of the disease and assess its severity.

He will then refer the patient to a hand specialist, who will decide which therapeutic approach to adopt.

To make the diagnosis, the Tubiana classification is used, named after its creator (Raoul Tubiana) and which allows the degree of contracture in flexion of each finger to be measured:

stage 0: no lesion
stage N: presence of nodule in the absence of finger flexion
stage 1: deformity in flexion between 0° and 45°.
stage 2: finger flexion deformity greater than 135°.

The angle is calculated as the sum of the flexion contracture angles of the joints of each radius.

Dupuytren’s syndrome is

mild, when it does not affect the gestures of daily life and does not require any intervention
moderate, when it is partially disabling and a first non-surgical therapeutic approach is necessary
severe, when it prevents common gestures and requires surgical intervention

Dupuytren’s syndrome, treatments

When Dupuytren’s syndrome is moderate, the doctor may prescribe

radiotherapy: ionising radiation is directed at the nodules and thickenings. The results are good, but you have to wait a few months to see them. In addition, there are various side effects: dry skin, flaking, thinning of the skin, susceptibility to malignant tumours (especially when the patient is irradiated for several days);
injections of collagenase (an enzyme that breaks down collagen into small pieces) into the thickened or nodular part: you have to wait 24 hours to see if it has taken effect. The most common side effects are swelling, burning, pain and bleeding (but sometimes nausea and headaches may also appear);
corticosteroid injections, to soften the nodules and reduce cell proliferation.

When the syndrome is serious and disabling, the only option is surgical treatment

percutaneous needle fasciotomy (or needle aponeurotomy) is performed on an outpatient basis under local anaesthesia. The surgeon sticks a very thin needle into the affected palmar area to separate the thickened connective tissue and thus straighten the fingers. Post-operative recovery is rapid and requires only a few sessions of physiotherapy; the operation is suitable for all categories of patients, but there is a 60% chance that symptoms will reappear;
palmar fasciotomy is also performed on an outpatient basis and under local anaesthesia, but it is more invasive as the palm of the hand is incised to separate the connective tissue with special instruments and to straighten the fingers. The chances of symptoms reappearing are very low, but recovery is longer (the patient has to wear a protective bandage and undergo physiotherapy afterwards) and the scar is noticeable;
fascectomy involves the total removal of connective tissue. It may be selective (only the affected tissue is removed), total (the entire palmar aponeurosis is removed) or consist of dermatofascectomy (both the aponeurosis and the skin covering it are removed). The operation is usually performed under general anaesthesia, with an overnight stay of at least one night, but it can also be performed under regional local anaesthesia (from the neck to the hand). The probability of the syndrome recurring is less than 10%, but scars remain visible, recovery times are long and physiotherapy sessions numerous.

However, surgery is not without complications.

Sometimes it is possible to experience

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skin tears, especially in the case of percutaneous needle fasciotomy
infections
joint stiffness (usually recoverable with physiotherapy)
haematomas in the palm of the hand, requiring drainage
evident scars
skin graft rejection
damage to the nerve endings of the fingers, which remain numb
complex regional pain syndrome (occurs in very rare cases and involves pain, stiffness and swelling in the hand)
loss of control of affected fingers (a very rare complication that may even require amputation)