Dupuytren Disease

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Dupuytren's contracture is an inherited disease that results in progressive fibrous tissue contracture of the palmar fascia.

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.

Risk Factors

• 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
• Smoking
• Alcohol consumption
• Diabetes - the disease tends to be milder with a slow progression

Clinical Features

History

The patient may report difficulties with manual activities and a palmar nodule.

Examination

The earliest sign is often a firm and thickened palmar nodule over teh 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.

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.

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.

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

Classification

There are three grades of Dupuytren's contracture

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

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

Treatment

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

Surgery

Surgery is indicated when there is metacarpophalangeal join contracture of 30 degrees, or if there is any degree of proximal interphalangeal joint contracture.

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.