Dupuytren contracture, a disease of the palmar fascia, results in the thickening and shortening of fibrous bands in the hands and fingers. This disease entity belongs to the group of fibromatoses that include plantar fibromatosis (Ledderhose disease), penile fibromatosis (Peyronie disease), and fibromatosis of the dorsal proximal interphalangeal (PIP) joints (Garrod nodes or knuckle pads). Guillaume Dupuytren received recognition for the condition that came to be associated with his name because of his expertise on the clinical findings, pathogenesis, prognosis, and treatment of this disease; however, Plater and Cline respectively provided the earliest known records of Dupuytren contracture and its surgical treatment (Verheyden, 1983).
Race, Sex, and Genetics
The incidence of Dupuytren contracture is highest in Caucasians, historically those of Celtic descent. Ling (1963) demonstrated that the prevalence of Dupuytren disease in a Celtic family was 24%, which increased to 74% with the inclusion of close relatives.
The disease affects men 7-15 times more often than it does women. Although the incidence of Dupuytren disease is higher in men, Hueston (1960) suggested that the male-to-female incidence rate may actually be less because the disease is less severe in women; therefore, this condition may go unnoticed until later in life. Most cases of Dupuytren disease occur in patients older than 50 years. Consequently, one may expect an increasing incidence as the aging population increases.
Dupuytren disease has long been known to be transmitted in an autosomal dominant fashion with variable penetrance. Neumuller et al (1994) demonstrated an increased relative risk of 2.94 for individuals who express human leukocyte antigen (HLA)-DR3.
The incidence of Dupuytren disease also increases with concurrent patient clinical conditions or factors such as diabetes, smoking, chronic alcoholism, seizures, and infection (Ross, 1999). (Rheumatoid arthritis is associated with a decreased incidence of Dupuytren contracture.) About 5% of individuals with Dupuytren disease are diabetic, with an increased prevalence that is proportional to the duration of the diabetes (Noble et al, 1984). One study showed a higher incidence of retinopathy and Dupuytren contracture in diabetic patients and attributes the cause to microangiopathic changes. Similarly, smoking is associated with microvascular changes that may contribute to Dupuytren disease (Burge et al, 1997).
The prevalence of Dupuytren disease in those with chronic alcoholism increases in proportion to the amount of alcohol consumed (Bradlow and Mowat, 1986). Although the incidence of Dupuytren disease is 2-3 times higher in individuals with epilepsy, opinions about the cause differ. One group of investigators concluded that electroencephalogram (EEG) abnormalities were more common in patients with Dupuytren disease than in those with other clinical conditions.
Another study showed a correlation between increased barbiturate medication and a higher occurrence of Dupuytren disease (Critchley et al, 1976), whereas other studies implicated a genetic link between the 2 diseases.
Lastly, there are conflicting results about whether the human immunodeficiency virus (HIV) is implicated in some cases of Dupuytren disease (Ross). Bower et al (1990) demonstrated an increased incidence in patients with both Dupuytren contracture and HIV infection, whereas a study by French researchers showed no statistically significant difference in these patients compared with the general population.
Although results of previous studies that linked repetitive manual labor to Dupuytren disease were inconclusive, more recent studies show that the incidence of the disease is 5.5 times higher with such labor. Anecdotal evidence suggests that Dupuytren disease occurs after trauma. Regardless of these results, a positive family history may play a role in both occupational and traumatic cases of Dupuytren disease.
Age and family history
Younger individuals with a positive family history for the Dupuytren disease have been reported, although the disease most often affects people older than 50 years. In addition, they often have other fibromatoses, such as plantar fibromatosis, knuckle pads, and penile fibromatosis, although this diathesis occurs in less than 1% of the patients with Dupuytren disease. Among patients affected with Dupuytren disease, only 10-20% develops knuckle pads, and about 10% develop plantar fibromatosis. Factors such as family history, age younger than 40 years, presence of related fibromatoses, and disease on the radial side of the hand indicate an increased severity of the disease and an increased likelihood of recurrence.
Dupuytren disease is an autosomal dominant fibroproliferative disease with variable penetrance. Therefore, the manifestation of this condition depends on genetic and environmental factors. Although Dupuytren contractures are associated with other diseases, such as diabetes, chronic alcoholism, and seizures, specific causal relationships have not yet been determined.
A number of growth factors, immunologic mediators, and free radicals are implicated in the development of Dupuytren contractures. Most likely, an inciting disease or event in a genetically predisposed individual causes a cascade of events that may include processes that promote the formation of growth factors and free radicals that ultimately leads to abnormal fibroproliferation and the appearance of the characteristic Garrod nodule. Even when homeostasis is ultimately achieved and fibroblastic growth lessens, the pathologic nodule and cord remain.
Investigators have proposed several hypotheses for the pathogenesis of Dupuytren disease. One cause may be localized ischemia and subsequent xanthine oxidase–derived free-radical formation from endothelial cells. Fibroblasts proliferate within the fascia, clustered around the microvasculature. Research has revealed that lower concentrations of free radicals cause fibroblast proliferation in laboratory cultures. Because active fibroblasts produce free radicals as well, the fibroblasts induce an autocrine positive-feedback effect on themselves, causing further ischemia to the microvasculature.
Research has shown that growth factors such as basic fibroblast growth factor (FGF), platelet-derived growth factor (PDGF), and transforming growth factor-beta (TGF-â) may signal the overproduction of the myofibroblasts and/or myofibroblastic activity of the fibroblasts. In addition, high levels of TGF–â may hinder apoptosis, or cell death, of the active myofibroblasts, unlike normal tissue healing. The increased concentration and activity of the myofibroblasts not only increase the total amount of collagen leading to the pathologic nodule but also cause remodeling of the normal collagen matrix and an increase in the ratio of type I collagen to type III collagen. Ultimately, the excess deposition of type III collagen and the formation of cross-links along the lines of tension on the palm and proximal digits result in contractures.
The typical patient with Dupuytren disease is aged 50 years or older and presents with a palmar nodule and cord adherent to the skin, as well as with a flexion contracture. The likelihood of Dupuytren disease also increases in patients with pitting over the nodule and/or nodules, which are present bilaterally.
Dupuytren disease must be distinguished from several other conditions that affect the hand, including trigger finger, stenosing tenosynovitis, a ganglion cyst, or a soft-tissue mass. Unlike Dupuytren contracture, trigger finger typically involves pain with flexion followed by the inability to extend the affected digit. Stenosing tenosynovitis may be distinguished from Dupuytren disease by pain and a history of overuse or trauma. A small, movable nodule that is tender to palpation at the metacarpophalangeal (MCP) joint is likely a ganglion cyst. A soft-tissue mass must also be excluded from the diagnosis, especially if the patient is significantly younger than the typical patient with Dupuytren disease and if he or she has no other risk factors.
A patient younger than age 40 years without involvement of the dorsal hand, foot, or penis is unlikely to have Dupuytren disease; however, the possibility of a sarcoma must be ruled out—although the pathologic findings of a biopsy will most likely reveal a benign etiology (eg, lipoma, inclusion cyst).
The basis of Dupuytren disease lies in the nodule and the cord, the pathologic counterparts to the tendon and pretendinous bands. Most often, a nodule forms on either side of the distal crease of the palm. Later, nodules may form near the MCP joint or next to the PIP joint of the thumb and fourth and fifth digits. Contractures may then form along the normal fascial structures.
In the palm, contractures occur in the pretendinous bands and natatory ligaments, which are subsequently called the pretendinous cord and natatory cord, respectively. In addition, a contracture may be formed by the attachment of the transverse fibers of the palmar aponeurosis, which is found at the crease between the index finger and the thumb. In the digits, normal fascial structures, including the volar superficial fascia and lateral digital sheets, effectively become the central cord and lateral cords, respectively.
The pathologic spiral cord arises from 4 anatomic components: the lateral digital sheet, the pretendinous ligament, the spiral band, and the Grayson ligament. Fascia deep to the neurovascular bundle forms the retrovascular cord. The spiral band is located on the lateral aspects of the digits and is named such because it spirals around the neurovascular bundle. As progressive fibrosis of the spiral band forms the spiral cord, the structure shortens and pulls the normally lateral neurovascular bundle toward the midline of the affected digit. Knowledge of the neurovascular bundle displacement is critical so as not to sever it during surgery.
In younger individuals in whom Dupuytren disease is suspected, epithelioid sarcomas and fibromas must first be ruled out.