Dupuytren Disease

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Written by: Dr Jeremy Steinberg – created: 16 June 2021; last modified: 17 April 2022

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Dupuytrens contracture.jpg
Grade 3 contracture of the right 4th finger.
Dupuytren Disease
Epidemiology prevalence 2% to 42%, more common in Northern European men.
Classification Nodules, cords, contractures
Treatment collagenase (not available in NZ), percutaneous fasciotomy, surgery
Prognosis Often progressive but unpredictable

Dupuytren's disease is a medical condition with a strong hereditary component that results in progressive fibrous tissue contracture of the palmar fascia. The most common affected location is the palm of the hand.

Aetiopathogenesis

It is believed to have an autosomal dominant inheritance pattern with variable penetrance. The genetic factors are not completely understood. Androgen receptors are found in Dupuytrens nodules, which may explain why it is more common in men. In diabetics it is thought that there are microvascular changes that cause ischaemic tissue damage leading to contracture.

There are several hypotheses as to the underlying pathophysiological mechanisms for causing disease. Local ischaemia may result in the production of xanthine oxidase free radicals which damage the perivascular connective tissue. There may then be a reparative process with the stimulation of fibroblast and myofibroblast activity. There is an alteration in the proportion of collagen, with type 1 being replaced by type 3, similar to that found in the proliferative phase of wound healing.

  • Stage 1: Proliferative stage, with the development of nodules over the MCPJs and PIPJs
  • Stage 2: Involutional stage with nodule contraction and collagen production increases with myofibroblasts becoming predominant and nodule-cord units develop.
  • Stage 3: Residual phase with reduction in myofibroblasts and hypocellular nodules and cords formed by types I and III collagen

Risk Factors

The largest risk factor is genetic, but some people are carriers. There is thought to be some sort of trigger leading to the manifestation of disease.

  • Men - twice as common as women
  • Ethnicity - Higher in those of European descent, especially Northern European descent. Those in Scotland, Iceland, and Norway have the highest prevalence. It has the pseudonym of "Viking disease."
  • Age over 40. It usually presents in the mid 50s for men, and mid 60s for women.
  • Smoking
  • Alcohol consumption
  • Diabetes - the disease tends to be milder with a slow progression

Classification

There are three grades of Dupuytren disease

  • Grade 1: thickened nodule and a band in the palmar aponeurosis that may progress to tethering, puckering, or pitting
  • Grade 2: peritendinous cord (band) with limited extension of the affected finger
  • Grade 3: presence of flexion contracture. This is called Dupuytren contracture

Anatomy

The anatomy of the hand is very complex as is the anatomy of which structures are involved in Dupuytren disease. The collagenous structures are three dimensional, extending from under the skin surface to bone. The structures are involved in anchoring the skin to the palm of the hand. See this video for a review of the anatomy.

Clinical Features

History

The patient may report difficulties with manual activities and a palmar nodule. They may report difficulty removing their hand from their pocket without it catching, difficulty releasing things from their hand, and impact on work due to difficulty in placing their hand flat on a table.

Examination

Hueston ‘table top’ test confirming contracture.[1]

The earliest sign is often a firm and thickened palmar nodule over the metacarpal head at the level of the distal palmar crease proximal to the MCP joint. There may be an associated band in the palmar aponeurosis. In later disease there is palmar skin change with thickening of the skin, tethering, puckering, pitting, and subcutaneous fat fibrosis. It usually starts on the palm of the hand and progresses distally.

Pretendinous cords tends to occur as individual nodules coalesce together. However nodules and cords can be present at the same time. With progression of the cords they can extend across the MCP joint and produce MCP joint flexion contractures leading to reduced extension of the affected digit. Cords that cross the PIP joint can cause PIP joint contractures.

The 4th digit is the most commonly affected, followed by the 5th, 1st, 3rd, and 2nd digits. Bilateral disease is commonly found. Usually one hand is more severely affected, and this is not related to hand-dominance.

The Hueston table top test can be used to assess for contracture. The patient lays their hand palm down on a tabletop and attempts to flatten their hand. If they are able to flatten their hand then no contracture is present. The test is positive if they cannot place their hand in a flat position.

Assess the degree of MCPJ and PIPJ contracture. For MCPJ contracture measurement, passively extend the PIPJ. For PIPJ contracture measurement, hold the MCPJ in flexion which relaxes the cord.

Ectopic disease can occur. Garrod's nodes are present in around one half of affected individuals. These are also known as knuckle pads. They refer to subcutaneous fibrosis on the dorsal aspect of the PIP joints. Garrod's nodes represents systemic fascial disease, and predicts bilateral involvement.

There may be involvement of the plantar surface of the feet - called Ledderhose's disease, and penis - called Peyronie's disease.

Dupuytren's diathesis refers to those with severe disease. These patients are usually young, and have rapid disease progression of the bilateral hands. These patients are more likely to have systemic disease.

Investigations

Ultrasound of the hand shows a mass lying between the flexor tendon and skin. Imaging has a limited role as the diagnosis is predominantly made clinically.

Differential Diagnosis

Differential Diagnosis
  • Trigger Finger - finger can be fully extended with a click
  • Epithelioid sarcoma - usually progressive and extends beyond the localised digits.
  • Camptodactyly - 5th digit contracture from an early age
  • Traumatic finger contracture - history of trauma
  • Callus

Treatment

The main goal of treatment is to improve digit extension and hand function. Treatment is usually recommended before hand function is severely affected. Severe finger contractions can lead to joint stiffness and a reduced chance of success with interventions. The condition is benign and so treatment isn't always necessarily required.[1]

Non-Interventional Treatment

Splints, physiotherapy, and steroid injections do not appear to be effective.

Enzymatic Fasciotomy

The research has mostly been done on collagenase clostridium (Xiaflex), however the distribution of this product in New Zealand was discontinued in 2019 due to a business decision (see here). It appears to have also been discontinued in other non-US countries.

Collagenase clostridium histolyticum lyses collagen and disrupts the cord. The day after the injection, manipulation of the cord is done to rupture it. The injection is done by inserting the needle perpendicularly in the cord. The finger is moved to confirm that the needle isn't inserted into the flexor tendon. After depositing one third of the volume, the needle is repositioned distally and proximally to inject the remaining volume. [1]

The recurrence rate is 47%. The complication rate is 96.6% vs 21.2% for placebo. Most complications are mild to moderate. These include bruising, injection-site haemorrhage, pain, upper limb pain, tenderness, ecchymosis, pruritus, swelling, skin lacerations, lymph node enlargement, erythema, and blisters.[1]

Percutaneous Needle Fasciotomy

A needle is used to divide the cord. The recurrence rate is up to 75%, and there is a risk of tendon and neurovascular injury. One modification is the addition of lipofilling to address the subdermal fat deficiency found in the condition.[1]

When comparing collagenase to needle fasciotomy the results are similar for MCP joint contracture. Collagenase has a higher haematoma risk, skin ruptures when they occur tend to be larger, and greater pain.

Surgery

Surgery is indicated when there is MCP joint contracture of >30°, or for PIP joint contracture of >15° (and for practical purposes before the MCP contracture reaches 90° and before the PIP contracture reaches 30°). This level of contracture is commonly disabling. Surgical treatment is generally the only proven treatment.[1]

  • Limited fasciectomy: the cords are removed. Recurrence rate is 20% at 5 years.
  • Dermofasciectomy: more extensive, removes all diseased tissue including subcutaneous fat and palmar skin. The wound is healed by secondary intension or covered with a full thickness skin graft.
  • Staged procedure: for severe PIP contractures. An external fixator is placed, with tension progressively increased over 6 weeks. The second stage is fasciectomy or dermofasciectomy. The fixator is removed after wound healing
  • Amputation and arthrodesis. For severe PIP contractures.

Post-operatively hand therapy is required and it can take several months for hand function to return to normal. The wounds can be tender during recovery. Up to 1 in 7 patients develop a postoperative flare reaction manifesting as swelling of the joints of the hand and difficulty in making a fist. Oral prednisone can be used in this case.

Prognosis

Dupuytren's contracture is a progressive disease in most cases. 75% of patients develop advanced disease. 10% will regress. Risk factors for more rapid progression are male gender, age under 50, smoking, and alcohol use.

Videos

External Sources

References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 Grazina et al.. Dupuytren's disease: where do we stand?. EFORT open reviews 2019. 4:63-69. PMID: 30931150. DOI. Full Text.

Literature Review