Dupuytren Contracture

@PATOMAUZ

INTRODUCTION

Dupuytren contracture (DC) is a progressive fibroproliferative disorder affecting the palmar fascia, leading to fascial thickening, nodule/cord formation, and eventual contracture with an inability to fully extend the finger(s). DC typically affects the third, fourth, and fifth digits and is common in men of northern European descent. DC tends to progress slowly over years/decades, although the degree of severity and functional impairment varies among individuals.

PATHOPHYSIOLOGY

Dupuytren contracture (DC) is marked by excessive fibroblast proliferation and collagen deposition (particularly type III collagen) within the superficial palmar fascia. Fibrosis is thought to be caused by overstimulation of the Wnt signaling pathway, which helps regulate fibroblast proliferation, polarity, and differentiation.

RISK FACTORS

Risk factors include age >50, male sex, family history, and Northern European ancestry. Manual work (eg, gardening) may also be a risk factor. DC occurs more commonly in patients with a history of tobacco and alcohol use and diabetes mellitus.

CLINICAL PRESENTATION

DC slowly progresses over years and presents initially with painless puckering of the skin just proximal to the metacarpophalangeal (MCP) joint. As fibrosis continues, pathognomonic fascial nodules form along the flexor tendons, which may be mildly painful initially (image). Fibrotic nodules eventually progress to palpable fibrotic cords, which eventually result in contractures that lead to decreased extension at the MCP and/or proximal interphalangeal (PIP) joints. The ring and little fingers are most commonly involved, with the middle finger involved to a lesser extent. Inflammatory features (eg, redness, swelling, significant pain) are absent. DC can affect one hand or both hands.

DIAGNOSIS

The diagnosis of Dupuytren contracture is made clinically based on the following chronic features in a patient with the inability to fully extend the finger(s):

Skin puckering (just proximal to the MCP joint)
Fascial nodules (along the flexor tendons)
Fibrotic cords (along the flexor tendons)
Contracture at the MCP and/or PIP joints

DIFFERENTIAL DIAGNOSIS

Trigger finger: is caused by thickening and inflammation of the pulley and/or flexor tendon near the MCP joint, creating a catching sensation as the finger is extended. Trigger finger presents with episodic difficulty and with finger extension associated with pain, whereas DC causes sustained difficulty with finger extension in the absence of pain.
Ganglion cyst: is a mucinous, gelatinous fluid filling the tendon sheath, presenting as mobile, rubbery nodules that do not lead to contractures of the hand.
Diabetic cheiroarthropathy (diabetic stiff hand syndrome): is characterized by the accumulation of abnormal collagen and presents in patients with long-standing diabetes mellitus who have joint stiffness; thickened, scleroderma-like skin; and sclerosis of the tendon sheaths. It can decrease extension at the MCP joints but typically involves all digits other than the thumb. Also, fibrotic nodules and cords, which are pathognomonic for DC, are not present.
Calcific peritendinitis: is an inflammatory condition caused by deposition of calcium hydroxyapatite crystals in the tendons. Because of the inflammatory nature of this condition, it presents with significant pain, erythema, and swelling, features absent in DC.
Flexor tenosynovitis: is inflammation of the tendons and their synovial sheaths, typically caused by bacterial infections. It is associated with significant pain, erythema, and swelling, features absent in DC.
Myositis ossificans: is characterized by heterotopic bone formation in muscles; this benign condition typically develops after contusion to large muscle groups (eg, quadriceps femoris), not small muscles of the hand. Patients often have pain in the affected muscle and a deep, palpable lump (ie, heterotopic bone).

MANAGEMENT

Treatment of DC aims to maintain hand function; options include both nonsurgical and surgical interventions.

Mild disease: Disease limited to fibrotic nodules (ie, without cords, which limits range of motion) can be observed. Hand tools can be padded to prevent the pain associated with gripping.
Persistent or progressive disease: Fibrotic nodules that are persistently painful or are rapidly enlarging can be treated with local injection of corticosteroid/lidocaine therapy, which can shrink the nodule.
Advanced disease: Patients with cords causing contractures require more aggressive therapy.Needle aponeurotomy is a minimally invasive procedure in which a needle is inserted percutaneously to divide the cord, thus releasing the contracture. This procedure is suitable for mild contractures.
Collagenase injection is a minimally invasive treatment that uses collagenase from Clostridium histolyticum to dissolve the fibrotic cord. After injection, manual manipulation to extend the finger(s) is performed to rupture the cord. This procedure is suitable for mild contractures.
Surgery is typically used to treat severe contractures. Cords can be transected (fasciotomy) or excised (fasciectomy).

COMPLICATIONS

When DC causes significant flexion contracture that has been present for a prolonged time, permanent and irreversible joint contracture can result.

PROGNOSIS

DC is a chronic condition that progresses at a variable rate, typically over years or decades. There is no cure. Recurrence after treatment is common, and retreatment may be warranted.

SUMMARY

Dupuytren contracture (DC) (table) is a progressive fibroproliferative disorder affecting the palmar fascia, leading to fascial thickening and fibrotic nodule/cord formation. Eventually, DC results in contractures with inability to fully extend the finger(s) at the MCP and/or PIP joint(s). The diagnosis is made clinically. Treatment of mild contractures includes needle aponeurotomy and collagenase injection, both of which disrupt the fibrotic cords and release the contractures. Severe cases typically require surgery to transect or excise the fibrotic cord.