Dupuytren's contracture

INTRODUCTION — 

Dupuytren's contracture is a relatively common disorder characterized by progressive fibrosis of the palmar fascia [1]. It is a benign, slowly progressive fibroproliferative disease of the palmar fascia. Initial fascial thickening is usually seen as a nodule in the palm, which can be painful or painless and often goes unnoticed and undiagnosed. Joint stiffness and a loss of full extension develop insidiously over a variable period of time but typically decades.

As the process evolves over years, nodules that are the first signs of the disease may progress to form longitudinal bands referred to as cords on the palmar fascia. These cords can contract, resulting in loss of extension of the metacarpophalangeal (MCP) joint (picture 1), proximal interphalangeal (PIP) joint, or both [2]. Other terms for this disorder include palmar fibromatosis and Dupuytren disease (DD), as the fingers are not always held in a fixed flexion deformity.

ETIOLOGY — 

The cause of Dupuytren's contracture is unknown; important factors include genetics, ethnicity, sex, and age and may include certain environmental factors and other diseases [3]. The disorder, which more commonly affects those of northern European ancestry, appears to have a pronounced genetic predisposition; 68 percent of male relatives of affected patients develop the disease. In a study involving patients from the Netherlands, Germany, and the United Kingdom, six of nine genetic loci found associated with genetic susceptibility to Dupuytren's disease contained genes encoding proteins in the Wnt-signaling pathway [4]. Overstimulation of this pathway, which can regulate cellular proliferation, could potentially lead to fibroblast proliferation and nodule formation in this disorder through effects upon beta-catenin [5]. However, the number of loci associated with Dupuytren's has expanded greatly over the past two decades and is approaching 100.

Pathologically, Dupuytren's contracture is characterized by fibroblastic proliferation and disorderly collagen deposition with fascial thickening. Formation of a nodule or nodules occurs in the early proliferative stage of the disease and is the pathognomonic lesion of Dupuytren's contracture. Nodules form due to proliferation of fibroblasts in the superficial palmar fascia and histologically are composed of fibroblasts and type III collagen. Smooth muscle fibroblasts and myofibroblasts are present in the nodules; increased concentrations of prostaglandins are also found within the nodules and may influence myofibroblast contractility [6]. The flexor tendons are not intrinsically involved, but invasion of the dermis occurs and results in characteristic puckering and tethering of the skin.

The presence of CD3-positive lymphocytes and the expression of major histocompatibility complex (MHC) class II proteins also suggest a possible role for a T-cell mediated autoimmune response in this disorder [7].

Dupuytren's contracture has been observed in association with the following conditions and habits [3,8-15]:

●Vibratory tools – A systematic review found that the incidence of Dupuytren's contracture is two to five times higher among workers exposed to repetitive handling tasks or vibration as compared with those not exposed such trauma, although this association remains controversial [8].

●Diabetes mellitus – Dupuytren's contracture occurs with increased frequency (16 to 42 percent) in adults with diabetes mellitus.

●Localized fibroses – Nodular plantar fibromatosis (Lederhosen's), nodular fasciitis of popliteal fascia, and Peyronie's disease have been associated [12,13]. (See "Peyronie's disease: Diagnosis and medical management".)

●Tobacco and alcohol use – Cigarette smoking and alcohol consumption have also been associated [14,15].

An association of Dupuytren's contracture with epilepsy and with the use of anticonvulsants has been proposed [16-18], but has not been confirmed in all studies [19]. Dupuytren's disease has also been associated with increased mortality, particularly in patients who develop the disorder prior to age 60 [20].

EPIDEMIOLOGY — 

Most patients present over the age of 50 and the prevalence increases with age. Dupuytren's contracture occurs more often in White males and is uncommon in people of African or Asian descent [21]. Approximately 80 percent of patients are male, although the sex ratio varies between populations, ranging from 3:1 to 9.5:1 [22]. Between 5 and 15 percent of males older than 50 years are affected [23]. Symptom onset is earlier in males, and their disease may be more severe with more rapid progression. The relative frequency of patients who are female increases with advancing age [22,24].

PRESENTATION — 

Patients typically first notice a thickening or a nodule in the palm that can initially be painful or painless, and loss of motion of the affected finger or fingers is experienced years later in the disease course. The patient often rubs the palm and fingers in an attempt to straighten them out when describing the condition. The cords or contractures are usually painless; on rare occasions, concurrent tenosynovitis (inflammation of tendon sheath) occurs and is painful. The ring and little finger are more commonly involved and are usually affected earlier than other areas of the hand. The third finger is less often affected, and the index finger and thumb are least commonly involved.

The rate of progression from early disease with a nodule is variable but typically occurs over years to decades. There is limited literature addressing the average time to an indication to intervene.

Two studies have indicated that plantar fibrosis, knuckle pads, and radial side (thumb and index finger) involvement have a stronger influence on recurrence and extension of Dupuytren's disease than bilateral hand involvement, little finger surgery, or early-onset disease [25,26].

DIAGNOSIS — 

The diagnosis of Dupuytren's contracture is clinical, based upon the history of painless loss of extension of the fingers and upon the following findings on physical examination:

●The earliest sign of Dupuytren's contracture is a triangular "puckering" of the dermal tissue over the flexor tendon just proximal to the flexor crease of the finger. Passive extension of the affected fingers will demonstrate the puckering of the skin as it courses over the metacarpophalangeal (MCP) joint.

●Discrete nodules may be visible and palpable along the course of the flexor tendons near the distal palmar crease (picture 1 and picture 2).

●The presence of a palpable cord running longitudinally in the subcutaneous tissue which puckers the skin and limits extension of the metacarpophalangeal (MCP) or proximal interphalangeal (PIP) joint (picture 3).

●Mild tenderness may be present over the nodule but usually resolves over several months.

●The nodule typically progresses over a variable period of time to form a fibrous cord that extends from palm into the digits, causing a flexion contracture of the MCP joint that is permanent and irreversible. When nodules or cords originate in the digits, these lead to PIP joint contractures.

●Signs of inflammation are absent in most cases; local tenderness, swelling, and pain with passive flexion and extension are absent unless concurrent tenosynovitis is present.

Soft tissue pads over the PIP joints on the extensor surface (knuckle pads) may be associated findings. Nodular lesions within the plantar fascia of the feet can develop concurrently.

Plain radiographs of the hand are not necessary. Calcification of the tendons or pathologic tissue/cords does not occur.

DIFFERENTIAL DIAGNOSIS — 

Dupuytren's contracture should be distinguished from diabetic cheiroarthropathy (limited joint mobility), palmar fasciitis (also referred to as palmar fibromatosis), camptodactyly, traumatic scars, Volkmann's ischemic contracture, and intrinsic joint disease. Diabetic cheiroarthropathy in patients with diabetes involves all fingers except the thumb, whereas Dupuytren's contracture more commonly affects just the fourth and fifth digits, with the other digits typically spared. In addition, the taut bands or cords and nodules characteristic of Dupuytren's contracture are not commonly seen with cheiroarthropathy. (See "Limited joint mobility in diabetes mellitus".)

Likewise, palmar fasciitis causes progressive flexion contractures of all the fingers of both hands due to tethering of the flexor tendons to the palmar fascia and skin; although a much rarer disorder, it is an important consideration because the palmar fascial fibrosis is frequently associated with a malignant neoplasm. Typically, palmar fasciitis presents with loss of flexion and extension in most/all digits and may present with polyarthritis, as opposed to Dupuytren disease, which presents with loss of extension only. (See "Malignancy and rheumatic disorders", section on 'Palmar fasciitis and polyarthritis syndrome'.)

Stenosing flexor tenosynovitis, or trigger finger, can be confused with Dupuytren's contracture but is usually easily distinguished as trigger finger shows a dynamic flexion contracture of one or more fingers that corrects with a distinct snap or "trigger" which is typically painful, while Dupuytren's contracture is characterized by a fixed flexion contracture that cannot be corrected. (See "Trigger finger (stenosing flexor tenosynovitis)".)

TREATMENT — 

The goals of treatment are to restore finger motion and to evaluate the need for surgery or other interventions. The therapy chosen depends upon the disease severity.

Mild disease — Patients with mild symptoms from nodules early in the disease may benefit from modifying tools (eg, by means of built-up handles using pipe insulation or cushion tape) and, when possible, by use of a glove with padding across the palm during heavy grasping tasks. The symptoms from nodules will usually subside spontaneously over a few months.

There are limited data to indicate that massage, splinting, or exercise will prevent the progression of the contracture [27]. Most clinicians who treat this condition believe there is no beneficial effect to these modalities in preventing contracture progression. A theoretical concern regarding stretching is raised by laboratory studies suggesting that fibroblasts and myoblasts are upregulated by mechanical tension, as might result from stretching of the digits, potentially leading to worsening of contractures.

Persistent or progressive symptoms — Intralesional glucocorticoid injection with triamcinolone acetonide and lidocaine hydrochloride may be helpful if local tenderness is bothersome (eg, the patient develops tenosynovitis or the nodule is persistently tender) or if the palmar nodule is growing rapidly (picture 4). In one study, an average of 3.2 local injections of glucocorticoids led to significant nodule regression, but complications including atrophy at the injection site or rupture of the flexor tendon are reported. There are no data indicating an impact of corticosteroid injection on disease progression [28]. Glucocorticoid injection is considered helpful only in the rare patient with painful nodules and persistent pain beyond the expected timeframe; cords with or without significant contracture do not respond to glucocorticoid injection.

Flexion contractures — Patients who develop flexion contractures of their fingers may benefit from more aggressive forms of therapy, such as surgery (open fasciectomy), percutaneous needle aponeurotomy, or collagenase injection.

Selection of therapy — The choice among available therapies for flexion contractures related to Dupuytren's should be based on the patient's preference after a discussion of the risks and benefits of each approach. Open fasciectomy leads to longer-lasting improvement in contractures compared with other types of interventions [29] but also carries a higher complication rate and has a longer recovery time [30]. Patients who prioritize long-term outcomes over short-term morbidity may therefore prefer this option, while those who have milder disease and/or prefer to avoid surgery may choose percutaneous needle aponeurotomy or collagenase injection.

Data directly comparing interventions for moderate to severe disease suggest that surgery may lead to better long-term outcomes with worse short-term disability:

●In a multicenter trial in Finland, 302 patients with Dupuytren's contracture were randomly assigned to treatment with one of three interventions: surgery, percutaneous needle aponeurotomy, or collagenase injection [29]. After three months, the success rates (defined as >50 percent contracture release and patient satisfaction with the outcome) were similar across the three treatment groups (71 to 73 percent); however, after two years, the success rate was highest for patients who had undergone surgery (78 percent), followed by those who had collagenase injection (65 percent) and then those receiving percutaneous needle aponeurotomy (50 percent).

●Another six-week trial compared percutaneous needle aponeurotomy with limited surgical fasciectomy for 166 metacarpophalangeal (MCP) contractures due to Dupuytren's [31]. Improvement in extension was greater in those who received the surgical fasciectomy, especially for those with more severe contractures; the passive extension deficit measured in degrees decreased by 79 versus 63 percent, respectively. Patient-reported satisfaction was similar in the two groups. However, disability was greater in those who received surgery.

●A third trial randomly assigned 672 patients with moderate Dupuytren's contracture to undergo collagenase injection or limited surgical fasciectomy [30]. Compared with collagenase injection, limited fasciectomy was associated with more favorable scores on the Patient Evaluation Measure-Hand Health Profile (PEM) at one year and fewer recurrences requiring repeat intervention. However, the rate of moderate to severe complications was 5 percent in the surgical group versus 2 percent in the collagenase group, with surgical complications including amputation, nerve injury, and infection.

Data comparing percutaneous needle aponeurotomy with collagenase injection are likewise limited but suggest similar outcomes. In a trial of 70 patients with Dupuytren's contracture, patients were randomly assigned to treatment with percutaneous needle aponeurotomy or collagenase injection [32]. After 30 days, the rate of successful correction was similar between groups but varied by joint, ranging from 50 to 67 percent for stage II proximal interphalangeal (PIP) contractures to 89 to 100 percent for MCP and stage I PIP joint contractures. Similarly, the recurrence rate did not differ between the treatment groups over three years of follow-up but did vary based on the type of joint involved (26 to 29 percent for MCP, 38 to 44 for stage I PIP, and 67 percent for stage II PIP contractures, respectively). The rate of transient adverse effects such as contusion and edema was higher for patients undergoing collagenase injection compared with percutaneous needle aponeurotomy (100 versus 15 percent); however, the only serious adverse effect reported (nerve damage) was in the percutaneous needle aponeurotomy group (6 percent).

Therapeutic options

Surgery — Surgery (open fasciectomy) has been most widely performed for advanced stages of disease, if function is impaired, or if a contracture is progressing. At present, typical interventions are a transection of cords (fasciotomy) or an excision of diseased fascial cords (fasciectomy) with or without excision of the overlying skin. Surgery (limited palmar fasciectomy) should be considered only with functional impairment or in the presence of a contracture that is progressive. The initial results are generally good, but the recurrence rate is high [33]. Flexion deformities >30 to 40 degrees at the MCP or >20 degrees at the PIP joint have been suggested as indications for surgery [33,34]. PIP joint contractures are less likely to achieve full correction of the contracture and are less likely to respond to surgery in advanced stages (eg, contractures >60 degrees). The aims of surgery are to reverse digital contractures and to restore hand function. Surgery in younger patients has a much higher recurrence rate than in older patients, but this is likely due to the increased biological virulence of the disease in patients who present with contractures at a younger age.

The likelihood of recurrence after surgery may be related to the degree of cellularity of the lesion. A study of 10 patients found that signal characteristics on magnetic resonance imaging (MRI) predicted cellularity and, thus, may provide prognostic information regarding the likelihood of recurrence after surgery, although further study is warranted before this is used in clinical practice [35]. A second operation can be performed in patients who have a recurrence after the first procedure, although the complication rates are higher [36].

The specific surgical technique used depends upon the individual characteristics of the patient and upon the preferences of the surgeon. More aggressive techniques such as radical fasciectomy or dermofasciectomy do not appear to offer an advantage over limited fasciectomies [37].

Percutaneous needle aponeurotomy — Percutaneous needle aponeurotomy, also referred to as percutaneous needle fasciotomy, is sometimes utilized as a less invasive procedure to treat Dupuytren's contracture. Data from one retrospective series suggest that percutaneous needling of the palmar or digital fascia results in improved extension of the involved MCP or PIP joints, but contractures often recur. This was the conclusion of a study of a series of 74 needle fasciotomies [38]. A much larger study of 1000 needle fasciotomies showed better results at the MCP joints compared with treatment at the PIP joints [39]. Substantial improvement (an average of 77 percent increase) was noted immediately after the needle fasciotomy, but a majority of contractures (65 percent) recurred within three to four years [38]. Among the other reported complications of this technique are nerve damage, pseudoaneurysm formation, and flexor tendon injuries [40].

In one study, patients treated with triamcinolone injection in combination with percutaneous aponeurotomy experienced a significantly greater maintenance of correction of flexion deformity at six months compared with those treated with percutaneous aponeurotomy alone [41]. Glucocorticoid injections alone have not proven to be beneficial for contractures [42].

Collagenase injection — Collagenase therapy can be used in patients with the same contractures as in patients who undergo surgery but who prefer a nonsurgical approach. The likelihood of full or nearly full correction of contracture is higher for patients with less severe contractures (<50 degree contracture) or with early-stage disease. It is effective in patients with larger contractures, but restoration of full extension is less likely. Its effectiveness in the treatment of recurrent disease is unknown.

The injection of Clostridium histolyticum collagenase into the Dupuytren's lesion was beneficial in an open-label study and in two randomized trials [43-45]. The largest trial randomly assigned 308 patients with fixed flexion contractures of ≥20 degrees at the MCP or PIP joints to receive up to three injections of collagenase C. histolyticum or placebo in the contracted collagen cord at 30-day intervals, with manipulation of the joints the day following injection [45]. After 30 days from the last injection, patients who received collagenase were much more likely than patients who received placebo to show improvements in the contracture to 0 to 5 degrees of full extension (64 versus 7 percent) and the mean range of motion (from 44 to 81 degrees versus 45 to 50 degrees). Response rates were better in patients with less severe contractures. Treatment-related adverse effects were more common in those receiving collagenase compared with those receiving placebo but were mostly limited to transient, local reactions (eg, peripheral edema in 73 percent, contusions in 51 percent, injection-site hemorrhage in 37 percent, and injection-site pain in 32 percent).

The rate of recurrence of contractures after collagenase injection appears modest. In a randomized trial, recurrence was observed in only 9 percent (5 of 62 joints) of successfully treated patients at the 24-month follow-up after the last injection [44]. However, longer-term (>5 years) follow-up in 1066 joints treated with collagenase demonstrated an overall recurrence rate of 47 percent. The recurrence rate was worse at the PIP joint (66 percent) than the MCP joint (39 percent), which parallels results seen with needle aponeurotomy and open surgery [46].

Two concurrent collagenase injections in the same hand addressing two joints or two fingers at the same time have been shown to be generally safe and efficacious [47,48]. However, one study found an increased rate of skin lacerations with concurrent collagenase injections compared with injection of a single joint [48].

Post-intervention care for all patients — Regardless of the type of intervention used, we ask patients to do post-treatment range of motion stretching exercises, with or without night splinting. This is based on the authors' clinical experience.

Other interventions — Prophylactic external beam radiation therapy can prevent progression and can provide symptomatic benefit in patients with mild to moderate flexion deformities; however, no controlled studies have been published [49]. In one series, 198 affected hands (most less than 45 degrees, but up to 90 degrees of flexion deformity) in 129 patients were irradiated [50]. Radiation was well-tolerated and was associated with a decrease in the mean number of cords, nodules, and skin changes. At 12 months, contractures regressed in over 50 percent and were stable in 37 percent, and only 2 percent required hand surgery for disease progression.

In a long-term follow-up (mean of 13 years) of early-stage Dupuytren's contracture treated with radiotherapy, more than 70 percent of patients remained stable or had regression of contractures in early stages (Tubiana stage N/I), while disease progressed in most patients (>60 percent) in stages I to II [51]. Most patients (66 percent) had long-term relief of local symptoms, such as burning sensations, itching, scratching, pressure, and tension. Radiotherapy did not increase the complication rate after surgery and was well-tolerated in most [51]. There was no evidence for a second malignancy induced by radiotherapy.

Other interventions, including continuous slow skeletal traction, dimethyl sulfoxide, vitamin E, allopurinol, physical therapy, ultrasound therapy, glucocorticoid injections, interferon, and splinting, have generally not been successful [42].

PROGNOSIS — 

Dupuytren's contracture runs a variable course. Some patients have little incapacity for many years, and regression occurs in approximately 10 percent [52]. In others, fibrous bands form and radiate distally so that, ultimately, the fingers are contracted by the taut bands and hand function is impaired. Without any therapy, progression is observed in about 50 percent of patients over six years of follow-up. All treatments are palliative; no therapy has been shown to stop the process.

INFORMATION FOR PATIENTS — 

UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Basics topics (see "Patient education: Dupuytren's contracture (The Basics)" and "Patient education: Hand pain (The Basics)")

SUMMARY AND RECOMMENDATIONS

●Definition and pathology – Dupuytren's contracture is a relatively common, benign, slowly progressive fibroproliferative disease of the palmar fascia. Initial fascial thickening is painless and often goes unnoticed and undiagnosed. As the process progresses, nodules form on the palmar fascia, and the finger gradually loses its flexibility, with contractures that draw one or more fingers into flexion at the metacarpophalangeal (MCP) joint or proximal interphalangeal (PIP) joint. Pathologically, Dupuytren's contracture is characterized by fibroblastic proliferation and disorderly collagen deposition with fascial thickening. (See 'Introduction' above and 'Etiology' above.)

●Etiology – The cause of Dupuytren's contracture is unknown. It exhibits a pronounced genetic predisposition and is more common in patients of northern European ancestry; genes encoding proteins in the Wnt-signaling pathway appear involved. Associated conditions include diabetes mellitus, the presence of other localized fibrosing conditions, cigarette smoking, alcohol consumption, and possibly repetitive hand use and vibratory trauma. (See 'Etiology' above.)

●Clinical presentation – Most patients present over the age of 50. The incidence increases with age, and about 5 to 15 percent of White males older than 50 years are affected. The relative frequency of female patients increases with advancing age. Patients typically complain of finger thickening or a nodule in the palm, or later loss of motion of the affected finger or fingers. The lesion is usually painless, although mild tenderness may be present over the nodules early in the disease course. (See 'Presentation' above.)

●Diagnosis and differential diagnosis – The diagnosis of Dupuytren's contracture is clinical, based upon the history of painless stiffness of the fingers and upon the presence of characteristic findings on physical examination. Dupuytren's contracture should be distinguished from diabetic cheiroarthropathy (limited joint mobility), palmar fasciitis, camptodactyly, traumatic scars, Volkmann's ischemic contracture, and intrinsic joint disease. (See 'Diagnosis' above and 'Differential diagnosis' above.)

●Treatment – The goals of treatment are to improve flexibility of the fingers and to evaluate the need for surgery or other interventions. The therapy chosen depends upon the severity of disease. (See 'Treatment' above and 'Mild disease' above.)

•Mild disease – Patients with mild symptoms from nodules early in the disease may benefit from modifying tools when performing certain tasks. (See 'Mild disease' above.)

•Persistent or progressive symptoms – Intralesional glucocorticoid injection with triamcinolone acetonide and lidocaine hydrochloride may be helpful if local tenderness is bothersome (eg, the patient develops tenosynovitis) or if the palmar nodule is growing rapidly. (See 'Persistent or progressive symptoms' above.)

•Flexion contractures – The choice among available therapies for flexion contractures related to Dupuytren's should be based on the patient's preference after a discussion of the risks and benefits of each approach. Open fasciectomy may be preferable for patients who prioritize long-term outcomes over short-term morbidity. Percutaneous needle aponeurotomy or collagenase injection are reasonable alternatives, especially when patients have milder disease or prefer to avoid surgery. (See 'Selection of therapy' above.)

Regardless of the treatment modality, all patients receive posttreatment range of motion stretching exercises, with or without night splinting.

●Prognosis – Dupuytren's contracture runs a variable course. Some patients have little incapacity for many years, and regression occurs in approximately 10 percent. In others, fibrous bands form and radiate distally so that, ultimately, the fingers are contracted by the taut cords and hand function is impaired. Without any therapy, progression is observed in about 50 percent of patients over six years of follow-up. (See 'Prognosis' above.)