DUPUYTREN'S DISEASE: causes, symptoms, diagnosis, therapy

Dupuytren's disease

What is Dupuytren's disease?

The disease of Dupuytren, or Dupuytren's contracture, is a pathology of the hand linked to the formation of fibrous tissue that thickens under the skin of the palm of the hand, leading to retraction of the tendons and partial flexion of the fingers.

Causes

The causes of Dupuytren they are not known. Numerous hypotheses have been advanced on its origin, it has been hypothesized that it is related to particular works or small trauma of the hand. In particular, in the past it was suspected that some devices, vibrating (for example pneumatic hammers) caused trauma to the tissues of the hand, from which then the thickening of the tissues originated. To date, it is believed that, rather than traumas, the disease is linked to abnormal tissue healing processes.

Dupuytren is linked tothickening of a fibrous tissue, there handheld band or palmar aponeurosis. It is a tendon-like structure normally found in the hand, just below the skin. It has a mechanical support function, just like the muscles and tendons of the body. In patients suffering from Dupuytren there is in the early stages the appearance of small hard nodules, which can be palpated immediately under the skin, and which originate from the palmar fascia.

These nodules they do not cause pain. They are composed of fibrous tissue, called collagen. In some rare cases these nodules can shrink in size and disappear on their own, but most of the time they become larger, thickening and forming real fibrous cords in the subcutis.

These cords increasing in size they retract, dragging with them the palmar fascia which is in contact with the tendons of the hand. Over time this can lead to bending of the fingers, which become flexed. Precisely for this reason we speak of "Contracture" of Dupuytren, even if it is a completely different problem from the much more famous muscle contractures, it concerns one tendon fascia.

It is a disease that can occur at any age, however it is much more common above the age of 50, and mainly affects men, where it tends to be more severe. It is recognized that genetics play an important role at the origin of the disease, with mechanisms that are not known today.

Symptoms

What are the main ones symptoms of the disease of Dupuytren? Early symptoms consist of the first appearance of a nodule palpable, hard, in the subcutis, which can gradually turn into a fibrous cord. The nodules do differentiate give her cyst of the but no because they have a hard, not soft consistency.

There skin of the hand may appear retracted above the lump, thinned, sometimes more shiny. The nodules and cords associated with this pathology do not cause pain. Dupuytren most frequently affects the skin of the Palm of the hand near the ring finger, or even at the little finger. More rarely, the middle and index may also be affected. The involvement of the thumb is very rare.

In advanced stages of disease, contracture manifests itself in flexion of the fingers. Initially this may be minor and not cause discomfort to the patient, although it is visible. If in the early stage the flexion affects only one finger, over time even neighboring fingers may be affected.

It could be said that patients have crooked fingers, flexed towards the palms. In the advanced stage of the disease, the forced flexion can become such that it greatly limits the movements of the hand, making it difficult or impossible to perform tasks that were previously performed without problems (grasping objects, playing instruments). Most of the time, only one hand is affected by the disease, however it is possible for the disease to occur in both hands. The set of symptoms reported above is said Dupuytren's syndrome.

There disease of Dupuytren is a so-called fibromatosis and can be associated with other diseases of the same type: for example plantar fibromatosis (disease of Ledderhose), similar problem, however, that concerns the sole of the foot, for which the patient has crooked toes, or the illnesses of Peyronie, which occurs only in men, with the formation of a fibrous cord at the level of the penis, which causes it to bend. Dupuytren must be differentiated from other hand disorders, such as cyst in the palm of the hand or Duplay's disease (shoulder-hand syndrome). Hand cysts do not cause the fingers to curve, unlike Dupuytren.

Diagnosis

There diagnosis of Dupuytren it is primarily clinical. The presence of nodules or fibrous cords in the palm of the hand in typical locations, and the flexion contracture of the fingers are a source of suspicion for this pathology. To better define the treatment it is useful to contact orthopedic specialists, hand surgeons or plastic surgeons. In the opinion of the latter, it is sometimes useful to perform additional tests, especially ultrasound of the hand MRI. This is not so much to confirm the diagnosis, but rather to define the relationships of the fibrous cords with the surrounding structures inside the hand, in preparation for a possible therapeutic intervention.

Therapy

The treatment of the disease of Dupuytren varies according to the clinical picture. It is usually initiated when flexion contracture occurs, this is because it is not certain that even in the presence of nodules or cords of tissue these necessarily retract causing flexion of the fingers. A patient with nodules or retracting cords could remain stable without the need for any type of therapy.

When contracture occurs, however, different types of treatment can be chosen.

Minimally invasive therapies

The most modern are shown below minimally invasive therapies:

  • Percutaneous fasciotomy with needle, it is a technique that can also be performed on an outpatient basis, which requires local anesthesia. It foresees to dissect the retracted fibrous cords as much as possible with the use of a large needle, through which anesthetic and cortisone are also injected, then proceed to manual re-extension of the finger. A dressing is applied for 24 hours, there is then no need for hand immobilization or physiotherapy, even if they can still be indicated by the specialist. It is a relatively new technique, still under study but showing promising results.
  • Percutaneous aponeurectomy with lipid graft. It is a similar technique to the previous one, the fibrous cords are dissected with a needle and with the same the retracting cord is separated from the overlying skin with which it is in contact. A small liposuction is also performed from the patient's abdominal fat, which is then re-injected at the level of the hand (maximum 10 ml of fat is injected). This serves to allow the separation of the fibrous cords from the skin and their healing. A brace must be worn for the week following the surgery.
  • Injection of collagenase, an enzyme synthesized by a bacterium, the clostridium histolyticum (bacterium of the same family to which the clostridium botulinum belongs, from which the known botulinum toxin used in many fields of medicine and plastic surgery is synthesized). Collagenase is injected into the affected hand, allowing the fibrous cords to dissolve. After 24 hours from the injection, the patient returns to the specialist for finger extension and manual rupture of the fibrous cord. It is then necessary to wear a special brace during the night for a few months, and carry out physiotherapy exercises of the hand.
  • Radiotherapy. There is little scientific evidence on its use, it is usually used only in the early stages of the disease.

In severe cases, with flexion contractures that severely restrict hand movements, micro-invasive techniques may not be effective enough. It may therefore be necessary to resort to surgery.

Surgical interventions

For the treatment of Dupuytren's syndrome surgery of the but no which provide for the palmar fascectomy, ie removal of the palmar fascia from which the disease originates. These can be more or less extensive, depending on the size of the fibrous cords. In some patients it is possible to just perform one partial fascectomy, removing only the portions of the palmar fascia corresponding to the affected fingers. In more advanced cases, it may be necessary to remove the entire palmar fascia by performing a complete fascectomy.

The reason why it is preferable to monitor patients with this pathology for a long time, intervening only when flexion of the fingers occurs, is because the disease of Dupuytren curtains to reappear often even after surgical therapy. There relapse it is more likely in patients with Dupuytren in both hands, or with Dupuytren and other fibromatoses such as plantar fibromatosis or Peronye's disease. This is due to the fact that these patients often have a genetic predisposition towards abnormal tissue healing. For the same reasons, today we tend to prefer micro invasive approaches in the treatment of this problem.

Great importance into avoid the relapses it is attributed to post-operative therapy. A physiotherapy with adequate exercises and the use of appropriate guardians, to guarantee the correct extension of the fingers, the stretching and elongation of the operated fibrous tissue and to avoid the appearance of relapses. It is also important to avoid overloading the hand, keeping it at rest and avoiding activities that involve gripping objects with force.

Other therapies

Among the other therapeutic aids used today are the laser therapy and the vitamin IS, however their effectiveness is not proven at the moment.

Bibliographic sources

  1. Bainbridge C, Dahlin L, Szczypa P, Cappelleri J, Guérin D, Gerber A, Current trends in the surgical management of Dupuytren's disease in Europe: an analysis of patient charts, Eur Orthop Traumatol. 2012 Mar; 3 (1): 31–41.
  2. Becker K, Tinschert S, Lienert A et al., The importance of genetic susceptibility in Dupuytren's disease, Clin Genet. 2015
  3. Pess, Gary M .; Pess, Rebecca M .; Pess, Rachel A. (2012). “Results of Needle Aponeurotomy for Dupuytren Contracture in over 1,000 Fingers”. The Journal of Hand Surgery. 37 (4): 651-6

 

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