eMedicine Specialties > Rheumatology > Soft Tissue and Regional Rheumatic Disease

Dupuytren Contracture

Author: Don R Revis Jr, MD, Consulting Staff, Department of Surgery, Division of Plastic and Reconstructive Surgery, University of Florida College of Medicine
Contributor Information and Disclosures

Updated: Nov 30, 2007

Introduction

Background

Dupuytren contracture is a benign, slowly progressive fibroproliferative disease of the palmar fascia that has no clear etiology or pathogenesis. Plater first described this condition in 1614, but Dupuytren presented a meticulous anatomical study in 1831 that established the palmar fascia as the site of origin, which distinguishes it from other causes of finger contracture. Dupuytren also devised an effective surgical approach through palmar fasciotomy.

Dupuytren contracture is most commonly observed in persons of northern European descent and may be severe enough to result in debilitating finger contractures. Most patients are affected bilaterally (65%); in unilateral cases, the right side is affected more often than the left. The most commonly involved digit is the ring finger, followed by the small finger, and then the middle fingers. The index finger and thumb are typically spared.

Although the etiology is unclear, 27-68% of patients report a positive family history, which provides strong evidence for a genetic influence. Males are more likely to be affected than females, and the symptoms of the disease are more severe in males. In some studies, manual laborers have a slight predominance of disease (45%) compared with non–manual laborers (41%). Risk factors include prior hand trauma (13%), alcoholism (10%), diabetes mellitus (8%), epilepsy (2%), cigarette smoking, myocardial infarction, and HIV infection.¹

Pathophysiology

The basic pathophysiology of Dupuytren contracture is fibroblast proliferation and collagen deposition. Why this uncontrolled proliferation of palmar fascia begins and why it continues to the point of debilitating flexion contractures remain unknown. Studies implicate growth factors, including basic fibroblast growth factor, platelet-derived growth factor, and transforming growth factor-beta.² These factors show increased expression within the diseased fascia.

The disease progresses in several stages. The proliferative stage is characterized by the development of a nodule or nodules, the pathognomonic lesion of Dupuytren contracture. Nodules are composed of fibroblasts and type III collagen. The proliferative stage is the most biologically active phase of disease. Multiple nodules are common and may be tender to palpation. They are often located near the distal palmar crease but may be found throughout the palm and even in the digits.

Once the nodules are well-established, the active contractile, or involutional, stage begins. This phase is a more advanced disease state, but it is somewhat less biologically active than the proliferative stage. Cords begin to develop proximal to the nodules, and grooves or pits in the skin denote skin fixation to the underlying fascia. During this phase, myofibroblasts replace fibroblasts as the predominant cell type; they show morphologic similarities to fibroblasts and smooth muscle cells. Myofibroblasts are capable of manufacturing collagen and causing contraction because they contain myofibrils within the cells. Myofibroblasts also have intercellular connections to each other, permitting the generation of synchronized contractile forces. These cells are found not only within palpable nodules and cords but also as individual cells or groups of cells throughout the entire palmar fascia.

Histologically, the involutional stage demonstrates a predominance of collagen types III and V and an accompanying expansion of extracellular matrix, particularly glycosaminoglycans. Collagen and glycosaminoglycan deposition is similar to that seen in healing wounds. Occurring throughout the palmar fascia, these changes mirror those observed in the active stages of tissue repair and remodeling and are not isolated in the cords and nodules. Whether the changes are partly responsible for the resulting contractures or are merely the result of the physical forces being applied to the palmar fascia is unknown.

Advanced disease is referred to as the residual stage, which is the most biologically quiescent phase. Nodules regress and metacarpophalangeal (MCP) joint or proximal interphalangeal (PIP) joint contractures become most severe. Tendonlike cords are visible and contain densely packed, inelastic, longitudinally oriented collagen fibers, predominantly type I. At this stage, relatively few cells remain.

Frequency

United States

Dupuytren contracture is common, reflecting immigration from northern Europe. Approximately 5-15% of males older than 50 years are affected.

International

In Norway, approximately 5.6% of individuals older than 60 years are affected. In Australia, 26% of males and 20% of females older than 60 years are affected.

Mortality/Morbidity

Morbidity is limited to lifestyle changes. MCP joint and PIP joint contractures may interfere with daily living activities, and lesions may be tender. Occasionally, Dupuytren contracture is associated with plantar fasciitis or Peyronie disease.

Race

Dupuytren contracture is very common in northern Europe and the United Kingdom and in countries inhabited by immigrants from these areas (eg, Australia, Canada, United States).

• It is less common in individuals of southern European descent.
• Dupuytren contracture is less common in blacks and Asians. Incidence in these populations is less than 3%. Disease in these individuals more often involves the palm rather than the digits; therefore, it is less likely to be clinically significant or to cause the patient to seek medical attention. Consequently, incidence in these populations may be underreported.
• Dupuytren contracture is least common (incidence <1%) among Indians, Native Americans, and individuals of Hispanic descent.

Sex

Approximately 80% of affected individuals are male. This male predisposition is consistent throughout all countries and races studied.

Age

Incidence increases with age. See Frequency for related statistics.

Clinical

History

Obtain a thorough medical history when evaluating a patient thought to have Dupuytren contracture. Conditions possibly related to this disease include the following:

• Diabetes mellitus
• Alcoholism
• HIV infection
• Epilepsy
• Trauma
• Manual labor
• Cigarette smoking
• Previous myocardial infarction

Physical

Perform a thorough physical examination before focusing on the involved hand or hands. Important points to assess during physical examination of involved extremities include the following:

• Firm nodules that may be tender to palpation
• Cords proximal to the nodules that are usually painless
• Skin blanching upon active finger extension
• Atrophic grooves or pits in the skin, denoting adherence to the underlying fascia
• Tender knuckle pads over the dorsal aspect of the PIP joints: If present, aggressive disease is indicated.
• Presence of MCP or PIP joint contractures: Objectively measure and record degree of flexion contracture to investigate possible compensatory distal interphalangeal (DIP) joint hyperextension or DIP joint contracture.

Causes

• Dupuytren contracture is probably genetic in origin. Several studies suggest an autosomal dominant pattern of inheritance with variable penetrance.^3,4
• HLA-B7 and HLA-DR3 have been identified in a number of patients, suggesting a possible immunologic influence as well.
• Other authors suggest that Dupuytren contracture may result from an error in growth and regulation of the fibroblast, resulting from chromosomal changes similar to those seen in cells undergoing neoplastic change. Trisomy 8 abnormality has been identified in the fibroblasts excised from some patients. Nodules certainly display some features of a benign neoplasm.
• Because the changes occurring in the palmar fascia approximate the normal stages of wound repair and remodeling, but to an excessive and detrimental extent, another theory holds that this disease is a response to trauma. One observation suggests that the subdermal fat pads in the palm gradually disappear with age in the races most affected by this process. This regression may expose the underlying fascia to trauma. Repeated exposure to compression, stress, and other mechanical forces may trigger a reparative process in the palmar fascia. The reason for the progression to severe joint contractures remains unknown.

• Search for CME/CE on This Topic »

• Dupuytren Contracture (Physical Medicine and Rehabilitation)
• Dupuytren Contracture (Dermatology)
• Dupuytren Contracture (Orthopedic Surgery)
• Localized Fibrosing Disorders - Linear Scleroderma, Morphea, and Regional Fibrosis (Rheumatology)

• FDA Approves Biologic for Debilitating Hand Disease
• FDA Advisory Committee Recommends Approval of Xiaflex for Dupuytren's Contracture
• Collagenase Injection Effective for Dupuytren's Contracture