Chapter 107. Dupuytren Disease

A 53-year-old man presented with stiffness in his hands. He said his hands began to feel stiff several years ago, and now he finds that he cannot straighten many of his fingers (Figure 107-1). He delayed seeing a physician because he did not feel any pain in his hands. He recently began having difficulty holding his woodworking tools and wants to regain the function he has lost in his hands. The physician diagnosed him with Dupuytren contracture and discussed the disease with him along with his options for treatment.

Dupuytren contracture is a flexion contracture of one or more of the fingers in the hand. Patients develop a progressive thickening of the palmar fascia, which causes the fingers to bend in toward the palm and limits extension. Diagnosis is clinical and the palpable nodules in the palm are considered diagnostic. Treatment has historically been surgical, but a new nonsurgical treatment with a collagenase has been approved.

Dupuytren disease, Dupuytren contractures, palmar fibromatosis, morbus Dupuytren, Ledderhose disease.

• Dupuytren contracture is an autosomal dominant disease with incomplete penetrance (Figure 107-2).
• Higher prevalence among whites, particularly of Northern European descent. There is an increasing incidence related to aging.5
• More common in men than women (approximately 6:1).1,3
• Incidence in the United States is estimated to be approximately 3 per 10,000 adults with an estimated prevalence of 7%.
• Higher incidence in people who use tobacco and alcohol or who have diabetes mellitus or epilepsy.2

Dupuytren contractures form in three stages:

• Myofibroblasts in the palmar fascia proliferate to form nodules.
• Myofibroblasts then align along the lines of tension, forming cords.
• Tissue becomes acellular leaving thick cords of collagen that tighten resulting in flexion contractures at the metacarpal phalangeal joint, the proximal interphalangeal joint, and, occasionally, the distal interphalangeal joint.

• Tobacco use.
• Alcohol consumption.
• Epilepsy.
• Diabetes mellitus.
• Carpal tunnel syndrome.
• History of manual labor.
• History of hand injury.

Clinical Features

• Clinical diagnosis that is based on the history and physical examination.
• Patients complain of a slowly progressive tightness in the hands and a lack of the ability to fully extend their fingers.
• Typically painless.
• Examination findings—Nodules with flexion contractures are considered diagnostic, particularly in older white males; however, nodules may disappear late in the disease.2

Typical Distribution

• Can be either hand.
• More commonly seen in the fourth and fifth digits (Figure 107-3).

Laboratory Testing

• Not indicated.

Imaging

• MRI of the contractures may be helpful prior to surgical intervention, but is not needed to confirm a clinical diagnosis.

Biopsy

• Typically not indicated.
• Early diagnosis or diagnosis in atypical populations, such as children, may require histologic confirmation.

Consider the other causes of hand contractures and palmar nodules including:

• Intrinsic joint contractures—Loss of range of motion from any primary joint disease.
• Trigger finger, stenosing tenosynovitis—Localized swelling of the flexor tendon limits movement within the sheath with resulting “triggering”; digit catches, but can be straightened.
• Rheumatoid arthritis—Bony deformities resulting in ulnar deviation at the metacarpophalangeal joints and/or the wrist.
• Ganglion cysts and palmar nodules.
• Occupational hyperkeratosis and callous formation.
• Hand tumors including epithelioid sarcomas and soft-tissue giant cell tumors.

The treatment goal of Dupuytren contracture is to maintain or restore hand function by increasing range of motion at involved joints.

Nonpharmacologic

• Physical therapy with splinting does not seem to be helpful as a sole treatment. SOR C
• Radiation therapy has been used but there is little evidence to support it and significant potential side effects of the treatment. SOR C
• Hyperbaric oxygen is being studied with mixed results. SOR C

Medications

• Intralesional injection of corticosteroids is only mildly successful and may place the patient at risk for tendon rupture. SOR C
• Collagenase injection, a nonsurgical treatment, shows promise in phase II and phase III trials.4 SOR B

Referral for Surgery

• Surgical correction is considered when there is at least 30 degrees of contracture at the metacarpophalangeal (MCP) joint. SOR C
• Surgical fasciotomy decreases the degree of flexion deformity and results in modest improvements in hand function. Studies indicated that improvements in function are best correlated to changes at the proximal interphalangeal joint.5 SOR B

• Recurrence rate is related to the amount of fascia that is removed on surgery.
• There is an increased risk for recurrence with time.

• Postoperative follow-up should include hand therapy with a goal of increasing extension in the affected digits.

• Modifying risk factors (e.g., smoking, alcohol intake) known to contribute to the development of Dupuytren contracture is prudent, but are not shown to alter the course of the disease.
• After surgery, postoperative hand therapy may improve function and use of the hand. However, initial decreases in joint deformity and improvements in hand function may be lost over time.

Patient Resources

• PubMed Health. Dupuytren's Contracture—http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002213/.
• American Family Physician (AAFP). Dupuytren's Disease: What You Should Know—http://www.aafp.org/afp/2007/0701/p90.html.

Provider Resources

• Mayo Clinic. Dupuytren's Contracture—http://www.mayoclinic.com/health/dupuytrens-contracture/DS00732.
• American Family Physician (AAFP). Trojian TH, Chu SM. Dupuytren's disease: diagnosis and treatment Am Fam Physician. 2007;76(1):86-89—http://www.aafp.org/afp/2007/0701/p86.html.
• Medscape. Dupuytren Contracture—http://emedicine.medscape.com/article/329414.