Dupuytren’s Contracture (Vikings Disease) Causes and Treatment

Dupuytren’s contracture is a condition of the hands. It is not a disease but a symptom of an underlying disease.

The skin of the hand becomes thicker and harder. In the beginning, the thickened and hardened skin is not visible. It can only be felt with the other hand.

Dupuytren’s contracture is noticed only when the condition progresses and the person develops some functional impairment.

At this stage, the patient may not be able to wear gloves, shake hands, or perform tasks that require fine movements such as writing.

Dupuytren’s contracture is also called “Vikings Disease“. It is pronounced as “du-pwee-TRANZ”. It affects the palmar surface of the hand.

The overlying skin is normal in the beginning. As the disease progresses, the skin becomes thicker and harder and can become visible.

How Dupuytren’s Contracture affects your hand:

Initially, Dupuytren’s contracture is felt as a hardened palmar surface of the hand. Over months and years, the disease progresses, and the skin eventually becomes a thick cord. This pulls the fingers. The patient can not fully extend or open the hand.

Commonly, it affects the ring finger and the little finger. However, it can also affect the other fingers too especially the middle and the index finger.

The fascia of the palmar surface of the skin may become so hard that it is difficult for the patient to move the affected fingers.

Dupuytren Contracture commonly affects men who are 50 years of age or older. It is also common if another family member is also affected. Individuals of Northern European descent and alcoholics are commonly affected.

Dupuytren’s contracture Causes:

The condition has been associated with multiple medical conditions and is often seen by general practitioners, physicians, orthopedic surgeons, and general surgeons.

• Dupuytren’s Contracture (DC) and Diabetes Mellitus:

Diabetes has been strongly linked with Dupuytren’s contracture of the hands. The exact mechanism of the association between the two diseases is not known. However, the activation of certain inflammatory mediators and fibroblasts activating factors can be the possible linking mechanism.

In on systemic review, Dupuytren’s contracture was present in 31% of the study patients who had diabetes compared to only 14% of the control population [Ref].

Another uncommonly described entity is the “Diabetic Hand Syndrome”. This condition may encompass the following diseases of the hands:

• Dupuytren’s Contracture
• Limited mobility of the hand joints
• Trigger finger
• Carpel Tunnel Syndrome
• Reflex sympathetic dystrophy, and
• Charcot Neuroarthropathy

The condition is sometimes used synonymously with diabetic cheiroarthropathy, diabetic pseudoscleroderma, or scleredema.

When the patient has concomitant “frozen shoulder” or “Adhesive capsulitis” of the glenohumeral joint, it is then referred to as the “shoulder-hand syndrome”.

It has also been suggested that DC is more common in patients with long-standing diabetes and those who are on insulin.

• Dupuytren’s Contracture and Chronic liver disease:

Dupuytren’s Contracture has been linked with chronic liver disease. Although, the association with chronic liver disease, in general, is not very strong, however, it is said to be more common in patients with alcoholic liver disease.

Like all other conditions, where fibrosis is the main pathology, chronic liver with scarring has been associated with the condition.

In one systemic review, the prevalence of the disease was 22.3% in patients with the chronic liver disease compared to 9.7% in the control group. [Ref]

• Garrod’s knuckle pads:

Knuckle pads or Garrod’s pads are benign fat pads overlying the small joints of the hands. The condition has been linked with Dupuytren’s contracture in as much as 44- 54% of the patients.

• Plantar fibromatosis (Ledderhose disease):

Plantar fibromatosis is an uncommon, benign, proliferative disease of the plantar fascia. The disease can locally progress to cause functional disability.

It has been associated with Dupuytren’s contracture in 6-31% of the patients.

• Penile fibromatosis (Peyronie’s disease):

Penile fibromatosis or Peyronie’s disease is a chronic inflammatory and fibrotic condition that affects the penile shaft.

It causes pain, deformity and a change in the curvature of the penile shaft. In literature, 2-8% of the patients have concomitant Dupuytren’s Contracture.

• Epilepsy and Dupuytren’s Contracture:

Epilepsy has been associated with the development of Dupuytren’s contracture. Certain studies have associated it with the use of antiepileptic drugs, however, data is limited in this regard.

The prevalence of Dupuytren contracture in one systemic review was 40.3% in patients with epilepsy compared to 29.2% in the control group [Ref]

Risk factors of Dupuytren’s contracture:

People who are at a high risk of developing the disease include:

• Men are usually affected more than women and have a more severe disease.
• With age the incidence of the disease increases
• People of Northern European descent are at a higher risk of developing the disease compared to other ethnic groups
• People with a positive family history are at a much greater risk of developing the disease.
• Smokers are at a greater risk of developing the disease
• Alcoholics have a greater chance of developing the condition than non-alcoholics.

Grading of Dupuytren’s contracture:

DC is categorized into three different grades based on the degree of hand involvement.

Grade 1:

• Grade 1 disease is a thickened nodule and a band-like palmar aponeurosis. The band may further progress to pitting, puckering, or skin tethering.

Grade 2:

• In grade 2 disease, there is a peritendinous band and limitation in extending the finger

Grade 3:

• Grade 3 disease presents as flexion contracture.

Tubiana’s Staging of Dupuytren’s Contracture

Treatment of Dupuytren’s Contracture:

The commonly used treatment modalities are outlined here:

Dermatofasciectomy:

• This procedure is effective in severe disease. However, it is too costly, involves the need for skin grafting, has prolonged rehabilitation, and increased risk of complications.

Limited fasciectomy:

• This procedure is effective for moderate to severe disease. It removes the diseased fascia and lowers the risk of recurrence.
• This procedure is costly, may be associated with scarring, and requires prolonged rehabilitation.

Open fasciotomy:

• This procedure is moderately invasive, has fewer complication rates (primarily due to better visibility). However, the recurrence rate is higher as the diseased tissue is not excised.

Percutaneous needle aponeurotomy:

• This is an outpatient procedure and is minimally invasive. Patients recover quickly after the procedure and is less costly.
• However, it is less effective for severe disease and has a higher recurrence rate.

Collagenase Clostridium histolyticum (Xiaflex):

• This procedure is minimally invasive with a low risk of complications.
• It is less effective for severe disease, has a greater chance of recurrence, and is costly. Furthermore, it is usually done on a single band and requires repeated procedures.

Ref: Mella JR, Guo L, Hung V. Dupuytren’s contracture: An evidence based review. Annals of plastic surgery. 2018 Dec 1;81(6S):S97-101.

Complications of Dupuytren’s Contracture:

Dupuytren contracture can make certain tasks more difficult. The thumb and index fingers are not usually affected so many people don’t experience any inconvenience or disability when performing fine motor activities like writing.

As the disease progresses, however, it can affect your ability to open your hands fully, grasp large objects, or get your hand into tight places.