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, nodules may progress over years to form longitudinal bands referred to as cords on the palmar fascia, and the finger gradually loses extension, with contractures that draw one or more fingers into flexion at 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].

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]:

●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].

●Dupuytren's contracture occurs with increased frequency (16 to 42 percent) in adults with diabetes mellitus. (See "Overview of the musculoskeletal complications of diabetes mellitus".)

●Other localized fibroses such as nodular plantar fibromatosis, nodular fasciitis of popliteal fascia, and Peyronie's disease [12,13]. (See "Peyronie's disease: Diagnosis and medical management".)

●Cigarette smoking and alcohol consumption [14,15].

An association of Dupuytren's contracture with epilepsy and with the use of anticonvulsants has been proposed [16-18], but has not 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].

PRESENTATION — Most patients present over the age of 50, and the prevalence increases with age. It occurs primarily in White males and is uncommon in people of African or Asian descent [21]. Approximately 80 percent of patients are men, although the sex ratio varies between populations, and approximately 5 to 15 percent of males older than 50 years are affected [22]. Symptom onset is earlier in men, and their disease may be more severe, progressing more rapidly. The relative frequency of patients who are women increases with advancing age [23,24]. The rate of progression from early disease with a nodule is variable, but typically over years to decades, and there is limited literature addressing the average time to an indication to intervene.

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 ulnar side of both hands is involved in most patients, with the fourth and then fifth fingers most commonly affected and 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.

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 does 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. The nodules or cords may also originate in the digits, which can lead to PIP joint contracture.

●The MCP and PIP joint contractures can result in permanent, irreversible, progressive flexion contractures of the affected joints (picture 1).

●Signs of inflammation are absent in most cases; local tenderness, swelling, and pain with passive flexion and extension are absent unless a 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 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," 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 severity of disease.

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. In some patients, there was no long-term impact on disease progression [28]. Glucocorticoid injection is considered helpful only in patients with nodules; cords with or without significant contracture do not respond to glucocorticoid injection.

Flexion contractures — The standard treatments for flexion contractures are surgical (open fasciectomy), as well as percutaneous or open fasciotomy or needle fasciotomy. Clostridial collagenase injection has also shown benefit [29].

Surgery — Surgery has been the treatment of choice 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 [30]. Flexion deformities >30 to 40 degrees at the metacarpophalangeal (MCP) or >20 degrees at the proximal interphalangeal (PIP) joint have been suggested as indications for surgery [30,31]. 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 severity 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 [32]. A second operation can be performed in patients who have a recurrence after the first procedure, although the complication rates are higher [33].

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 [34].

Percutaneous needle aponeurotomy — Percutaneous needle aponeurotomy, previously 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 [35]. A much larger study of 1000 needle fasciotomies showed better results at the MCP joints compared with treatment at the PIP joints [36]. 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 [35]. Among the other reported complications of this technique are nerve damage, pseudoaneurysm formation, and flexor tendon injuries [37].

Limited data suggest that surgical fasciectomy is superior to percutaneous needle aponeurotomy in terms of the total increase in extension, but not in more subjective outcomes such as patient satisfaction and self-reported disability. When percutaneous needle aponeurotomy was directly compared with limited surgical fasciectomy in a six-week trial that randomly assigned 166 affected MCP fingers, improvement in extension was significantly greater in those who received the open-surgical fasciectomy; the passive extension deficit measured in degrees decreased by 79 versus 63 percent, respectively [38]. Patient-reported satisfaction was similar in the two groups, and disability was less in those receiving the percutaneous needle aponeurotomy. In this study, there were no complications of needle fasciotomy. Limited fasciectomy was especially superior in worse contractures (Tubiana stages III and IV). Percutaneous needle aponeurotomy may have some role in patients with milder disease (Tubiana stage I and II), because of quicker recovery and fewer complications.

Additionally, 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 [39].

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. Collagenase injection therapy is most effective 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 Clostridial histolyticum collagenase into the Dupuytren's lesion was beneficial in an open label study and in two randomized trials and may be an effective alternative to surgery [40-42]. 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 clostridium histolyticum or placebo in the contracted collagen cord at 30-day intervals, with manipulation of the joints the day following injection [42]. Cords injected with collagenase were significantly more likely to show a reduction in the contracture to 0 to 5 degrees of full extension 30 days after the last injection (64 versus 7 percent). The mean range of motion in the treated joints also improved significantly (from 44 to 81 degrees versus 45 to 50 degrees). Response rates were better in patients with less severe contractures.

Patients treated with collagenase injection experienced significantly more treatment-related adverse events compared with placebo. However, these were mostly limited to transient, local reactions. Patients who received collagenase injections developed peripheral edema (73 percent), contusions (51 percent), injection-site hemorrhage (37 percent), injection-site pain (32 percent), and upper extremity pain (31 percent). Few serious adverse events were noted among 204 collagenase-treated patients (two tendon ruptures and one case of complex regional pain syndrome). No systemic allergic reactions, nerve injuries, or changes in flexion or grip strength were seen. No recurrences were seen, but because follow-up was only for 30 days, no long term (six-month) recurrence information was determined.

In a similar, but smaller, 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 [41].

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 [29].

Two concurrent collagenase injections in the same hand have been shown to be generally safe and efficacious [43,44]. However, one study found an increased rate of skin lacerations with concurrent collagenase injections compared with injection of a single joint [44].

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 [45]. In one series, 198 affected hands (most less than 45 degrees, but up to 90 degrees of flexion deformity) in 129 patients were irradiated [46]. 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 [47]. 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 [47]. 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 [48].

PROGNOSIS — Dupuytren's contracture runs a variable course. Some patients have little incapacity for many years, and regression occurs in approximately 10 percent [49]. 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.

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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 standard treatments for moderate to severe flexion contractures are surgical (open fasciectomy), as well as percutaneous or open fasciotomy or needle aponeurotomy. Collagenase injection has also shown benefit. Regardless of the treatment modality, all patients receive posttreatment range of motion stretching exercises, with or without night splinting. (See 'Flexion contractures' above and 'Surgery' above and 'Percutaneous needle aponeurotomy' above and 'Collagenase injection' above.)

●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.)