Dupuytren Disease

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

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

There is a small lump or multiple lumps with pits in the palmar aspect of the hand. These progress to contractures of the fingers.

There may be involvement of the plantar surface of the feet and penis. Bilateral disease can occur. Garrod's nodes can be present.

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

Injections

• Intralesional corticosteroid injections
• Collagenase
• Percutaneous needle fasciotomy

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.