Practice Essentials

Dupuytren disease is a fibrosing disorder that results in slowly progressive thickening and
shorting of the palmar fascia and leads to debilitating digital contractures, particularly of the
metacarpophalangeal (MCP) joints or the proximal interphalangeal (PIP) joints. This
condition usually affects the fourth and fifth digits (the ring and small fingers). See the
images below.

Arrow denotes the cord often present in Dupuytren contracture. Metacarpophalangeal joint
and proximal interphalangeal joint contractures are also present.
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This photo shows a patient with an inability to extend the fourth and fifth digits. The
differential diagnosis includes Dupuytren contracture, which is a flexion contracture most
commonly involving digits 4 and/or 5.
View Media Gallery
Dupuytren contracture belongs to the group of fibromatoses that include plantar fibromatosis
(Ledderhose disease), penile fibromatosis (Peyronie disease), and fibromatosis of the dorsal
PIP joints (Garrod nodes or knuckle pads). [1] Although many cases appear to be idiopathic,
various associated diseases have been reported. See Pathogenesis/Etiology.
Dupuytren contracture is most commonly observed in persons of Northern European descent
and affects 4-6% of Caucasians worldwide. [2] Many individuals have bilateral disease (45%);
in unilateral cases, the right side is more often affected. [3] The ring finger is most commonly
involved, followed by the fifth digit and then the middle finger. The index finger and the
thumb are typically spared. See Epidemiology and Presentation.
Although the cause of Dupuytren disease is unknown, a family history is often present. Males
are three times as likely to develop disease and are more likely to have higher disease
severity. [4, 5] Male predominance may be related to expression of androgen receptors in
Dupuytren fascia. See Etiology and Epidemiology. [6]
Other potential risk factors include manual labor with vibration exposure, prior hand trauma,
alcoholism, smoking, diabetes mellitus, hyperlipidemia, Peyronie disease, and complex
regional pain syndrome. [7] Rheumatoid arthritis seems to protect against the development of
Dupuytren disease. See Etiology.
Therapies include conservative medical and surgical modalities. Although the condition is not
fatal, significant morbidity can occur if patients are untreated. See Treatment.
Stages of Dupuytren disease
Dupuytren disease occurs in the following three stages:
Proliferative phase - During this phase, myofibroblasts proliferate and a nodule
develops. In early disease, some patients may report tenderness and discomfort
associated with the nodules. The associated pain is thought to be due to nerve fibers
embedded in the fibrous tissue or compression of local nerves. [8] On physical
examination, palmar skin blanching is seen with finger extension.
Involutional phase - In this phase the disease, spreads along the fascia and into the
fingers resulting in the development of a cord. Myofibroblasts are the predominant cell
type during this phase and align themselves along tension lines within the nodule
Residual phase During the residual phase, the disease continues to spread into the
fingers and the cord tightens creating a contracture. The nodular tissue disappears as do
the myofibroblasts and acellular tissue with thick bands of collagen remain.
Dupuytren disease is not always progressive, however. A prospective study in 247 Dutch
participants with primary Dupuytren disease with follow-up at intervals of 3 to 6 months
found that in up to 75% of patients, the disease stabilizes or even regresses. [9]
Grades of severity
The grading system for Dupuytren disease severity is as follows (see the images below) [10] :
Grade 1 - Thickened nodule and band in the palmar aponeurosis; may have associated
skin abnormalities
Grade 2 - Development of pretendinous and digital cords with limitation of finger
extension
Grade 3 - Presence of flexion contracture
See the images below.
Arrow denotes the cord often present in Dupuytren contracture. Metacarpophalangeal joint
and proximal interphalangeal joint contractures are also present.
View Media Gallery

Arrow denotes the typical cords of Dupuytren contracture. These cords are usually painless.
Note the metacarpophalangeal joint contracture.
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This photo demonstrates the presence of a nodule as well as skin blanching with extension of
the affected digits.
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Three clinical grades of Dupuytren disease.

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Anatomy
In the normal hand, the palmar aponeurosis runs longitudinally from the wrist, crosses over
the superficial transverse palmar ligament, and splits into pretendinous bands to each digit.
See the image below.
 Normal anatomy of digital ligaments.
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In the distal palm and fingers, the following superficial fascial components are typically
involved in Dupuytren disease:
Pretendinous band
Spiral band
Lateral digital sheet
Grayson ligament
Natatory ligament
The extension of a palmar fascial band to the index finger frequently ends in the skin on the
radial side of the hand. The band to the thumb is inconsistent. The insertion of the
pretendinous bands to the skin distal to the distal palmar crease is by means of a bifurcate
insertion into the side of the finger dorsal to the neurovascular bundle. A natatory ligament
runs transversely across each web space distal to the MCP joint, giving fibers that blend with
each lateral digital sheet and to the superficial aspect of the flexor tendon sheath. The
superficial transverse ligament lies deep to the pretendinous bands, proximal to the MCP
joints and the natatory ligament. In the fingers, the Cleland ligament, Landsmeer ligaments
(oblique retinacular ligaments), and other deeper fascial layers are usually spared in
Dupuytren disease. [11, 12, 13]
According to Luck, normal longitudinal components of the superficial palmar aponeurosis are
referred to as bands; diseased tissue is referred to as cords. [14]Cardinal features of Dupuytren
disease are the nodule, the cord, and the digital flexion contracture. The bands and cords are
characterized as follows:
The pretendinous cord is formed from pretendinous bands.
The spiral cord is made up of the pretendinous band, spiral band, lateral digital sheet,
and Grayson ligament; this often occurs in the ring and small fingers and winds around
the neurovascular bundle.
The lateral cord is formed from the lateral digital band and is rarely observed, except on
the ulnar aspect of the small finger.
 The central cord has no defined fascial precursor; it is the most common cause of
proximal PIP contracture.
The natatory cord contributes to web space contractures and passes superficial to
neurovascular bundles.
See the images below.

Visible cord characteristic of Dupuytren disease with planned markings for surgical release.
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Dissection of a diseased cord.

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 Parts of the palmar and digital fascia that become diseased in Dupuytren disease (left).
Diseased fascia that is associated with the pretendinous cord (center). Diseased fascia that is
not associated with the pretendinous cord (right).
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Normal parts of the fascia that produce the spiral cord (left). The spiral cord demonstrating
medial displacement of the neurovascular bundle in Dupuytren disease (right).
View Media Gallery
Spiral and lateral cords displace the neurovascular bundle toward the digital midline, while a
central cord may encase the neurovascular bundle and usually is directed toward one side of
the finger. The central, lateral, and spiral cords terminate on the tendon sheath of the adjacent
middle phalanx. One or more may be found in any individual patient, but they seldom occur
on both sides of the same finger. McFarlane describes displacement of the neurovascular
bundle superficially and toward the digital midline by a spiral cord, which makes it more
vulnerable to injury during surgery. [13] With increasing degrees of flexion contracture, nerve
compression and vascular embarrassment may also occur.
In the thumb, 3 fascial structures may be involved: the natatory ligament, the pretendinous
band, and the superficial transverse ligament of the palm. Knuckle pads may develop with
fibrosis in the dorsal subcutaneous wrinkle ligaments (of McGrouther) dorsally at the PIP
joints. These often indicate progressive disease.
Pathogenesis/Etiology
Although the underlying etiology for the development of Dupuytren disease is uncertain, the
basic pathophysiology involves fibroblast proliferation, and collagen deposition leading to
contractures of the palmar fascia. Investigators have proposed several hypotheses for the
pathogenesis of Dupuytren disease. Al-Qattan hypothesizes that an individual with a genetic
predisposition to develop Dupuytren disease experiences a second inciting event (ie,
smoking, diabetes, trauma, alcoholism), resulting in microvascular ischemia. [6] The localized
ischemia causes two events to take place in the palmar fascia: (1) the conversion of adenosine
triphosphate to hypoxanthine and (2) the conversion of xanthine dehydrogenase to xanthine
oxidase.
Xanthine oxidase acts as a catalyst for the oxidation of hypoxanthine to xanthine and uric
acid; this conversion results in the production of free radicals. Free radicals result in
fibroblast proliferation and the production of numerous cytokines. Interleukin 1 (IL-1) is the
most abundant cytokine in Dupuytren disease and, via its receptor, upregulates the production
of transforming growth factor beta (TGF-beta), fibroblast growth factor, epidermal-derived
growth factor, and platelet-derived growth factor. [15, 16] This milieu of cytokines and growth
factors results in the proliferation of fibroblasts and their differentiation into myofibroblasts
(the primary cell type in Dupuytren disease). [17]
Increased levels of nerve growth factor are present, which induce fibroblast transformation to
myofibroblasts, especially during stages II-III. The splicing of fibronectin affects collagen
binding. Platelet activation produces lysophosphatidic acid (LPA). LPA is a signaling
molecule for cell proliferation and myofibroblast contraction. After binding to its receptor on
myofibroblasts, LPA leads to a decrease in cyclic adenosine monophosphate and an increase
in intracellular calcium levels. These events mediate smooth muscle contraction of the
myofibrils within the myofibroblast. Normal palmar fascia is primarily composed of type I
collagen; Dupuytren disease is associated with an increase in type III collagen.
The ratio of type III collagen to type I collagen increases, the reverse of the normal pattern, in
the palmar fascia. [10] Excess type III collagen production occurs from an increased density of
fibroblasts secondary to enhanced stimulation and diminished apoptosis, as well as an
imbalance between the collagenases and their endogenous inhibitors. Dupuytren fascia
exhibits an increased ratio of tissue inhibitors of metalloproteinases (TIMP) to matrix
metalloproteinases (MMP). [18, 19, 20]
The myofibroblasts are indirectly connected to collagen, and the contractile force is
transmitted from the intracellular actin microfilaments to the collagen bundles. Ultimately,
the end result is contracture from excess deposition of type III collagen and the formation of
cross-links between myofibroblasts and collagen. Another hypothesis by Vi et al suggests an
up regulation of the gene transcript POSTN mRNA that encodes the protein periostin.
Periostin is secreted by diseased cord myofibroblasts into the extracellular matrix. It
promotes the transition of palmar fascia fibroblasts into a myofibroblast phenotype, thereby
enhancing disease progression. [21]
Additionally periostin is prevalent during the early stages of bone fracture repair and vascular
injury. Hueston advanced the extrinsic theory, suggesting that Dupuytrens nodules arise de
novo and progress to cords. [22] Vasomotor disturbance and neurovascular mediation by the
skin are possible contributors to the development of Dupuytren disease. In contrast,
McFarlane postulated the intrinsic theory, that the cords of more advanced Dupuytren disease
are derived from normal fascia. [13] A combination of these ideas forms the synthesis theory,
which states that the nodules and pretendinous cords represent different forms of the
disease. [23]
Genetic factors
Though the cause of Dupuytren contracture remains unknown, genetic factors are thought to
play a role. [24] One study derived a sibling recurrence-risk ratio of 2.9 (range, 2.6-3.3). [25]
Furthermore, DNA microarray analysis has demonstrated that the gene MafB is up-regulated
in Dupuytren cord tissue. MafB is involved in tissue development and cellular
differentiation. [26, 25] Further support for a genetic link was provided by Al-Qattan, who
described a maternally transmitted inheritance within the mitochondrial genome in 90% of
patients. The defective mitochondria generate high levels of free radicals and defective
apoptosis and are therefore directly related to disease pathogenesis. [6]
Another study of 20 patients with apparent maternal inheritance identified a polymorphism in
the mitochondrial 16s rRNA region present in 90% of their DNA. [27]Other studies suggest an
autosomal dominant pattern of inheritance with variable penetrance. [28] One such study
analyzed 5 generations of a Swedish family and mapped the affected gene (not yet identified)
to 16q. [29] Some authors suggest an error in growth and regulation of the fibroblast resulting
from chromosomal abnormalities, similar to those seen in cells undergoing neoplastic
changes. Trisomy 7 and 8 have been identified in the fibroblasts excised from some
patients.[30] Nodules may display features of a benign neoplasm. [31]
Others believe that Dupuytren contracture has a multifactorial inheritance, similar to diabetes
or hypertension.
Additional risk factors
Whether certain conditions represent independent risk factors for the development of
Dupuytren contracture is unclear. A large, retrospective study by Loos and colleagues on
2919 hands on which surgery had been performed revealed no statistically significant
evidence that the occurrence of Dupuytren contracture could be correlated with the presence
of diabetes, with alcoholism, or with smoking. [31]
Another report, however, found different results, noting an association between Dupuytren
contracture and several conditions (eg, alcoholism, diabetes, epilepsy, pulmonary disease), as
well as a link with smoking. [32] Nonetheless, even if such associations exist, no clear causal
relationship has been established in the literature.
HLA-DRB1*15 and HLA-DR3 have been identified in numerous patients, suggesting an
immunologic influence. Each confers about a 2-3 times relative risk for the development of
Dupuytren disease. [33, 34] 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.
Many hand surgeons believe that trauma to the hand or the distal part of the forearm, such as
falling on an outstretched hand, may precipitate the onset. [35] (A positive family history may
play a role in occupational and traumatic cases of Dupuytren disease.) However, numerous
population-based studies have failed to conclusively link Dupuytren disease to
trauma, [36] although case reports have suggested a possible association. Reports by Lucas et
al found increased Dupuytren disease in a group of French male civil servants with
occupational exposure to vibration and manual work. [37]
Case reports of Dupuytren disease occurring after surgical injury to the hand have been
identified, with the authors suggesting that injury can trigger the onset of Dupuytren disease.
A history of manual labor with vibration exposure or recurrent trauma has been found to
result in a 5-fold increase in the incidence of Dupuytren disease. [38, 39, 40] Liss performed a
systemic review of the literature regarding occupation and Dupuytren disease. Although he
failed to identify a link between trauma and development of disease, he did find an
association between occupational exposure to vibration and Dupuytren disease. [36]
Notably, the exposure in one study was severe enough to cause persistent symptomatic
circulatory disturbance ("vibration white finger") [38] ; in another, the exposure was associated
with other soft tissue "wasting" and peripheral nerve damage. In a review of 46 available
studies, Melhom and Ackerman concluded evidence suggests an association between
Dupuytren disease and vibration but not with highly repetitive or forceful work. [41]
Diabetes mellitus has been identified as a risk factor. [42] Dupuytren disease in patients with
diabetes ranges from 1.6-32%, the prevalence of diabetes in patients with Dupuytren disease
is 5%. [4] The use of insulin and oral hypoglycemics are strongly associated with Dupuytren
disease. The disease seems to occur at a younger age and tends to be more severe in those
with type 1 diabetes. [43]
Patients with diabetes mellitus and Dupuytren disease have a 4-fold increased risk of
developing microalbuminuria than do persons with diabetes alone. [44] A higher incidence of
retinopathy was identified in diabetics with Dupuytren contracture, likely attributable to
microangiopathic changes. Alcoholic liver disease Individuals with alcohol-related liver
disease have an increased prevalence of Dupuytren contracture (approximately 20%)
compared with control populations. Patients with liver disease from other causes do not
appear to be at increased risk. The reason for this is unknown, and some studies have
disputed the association. [45]
A 3-fold increased risk for Dupuytren contracture is seen in individuals who smoke, even
when studies control for alcohol use, perhaps due to microvascular
impairment. [32] Autoantibodies to connective tissue Significant associations have been found
with HLA-DRB1*15, HLA-DR3 and autoantibodies to collagen types I-IV have been
reported. [46]
Two studies have shown increased sensitivity to androgens in the palmar fascia. [47,48] This
may account for the male predominance of the disease.
Although implicated in previous studies, little conclusive evidence has been reported to link
epilepsy and antiepileptic medications to the development of Dupuytren contracture.
Phenobarbitone, in particular, results in increased LPA levels. 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 persons with other clinical
conditions.
Another study showed a correlation between increased barbiturate medication and a higher
occurrence of Dupuytren disease, whereas other studies implicated a genetic link between the
two diseases. [49]
Results are conflicting as to whether the human immunodeficiency virus (HIV) is implicated
in some cases of Dupuytren disease. [50] Bower et al demonstrated an increased incidence in
patients with both Dupuytren contracture and HIV infection, whereas a study by French et al
showed no statistically significant difference in these patients compared with the general
population. [51]
Epidemiology
Frequency
Dupuytren disease is common in the United States with a prevalence of 4%, reflecting
immigration from Northern Europe. [10] In Northern Europe the prevalence ranges from 4-
39%. In Norwegian populations, 30% of males over the age of 60 years are affected. The
 incidence of Dupuytren disease for the British population in 2004 was calculated at 34.4 per
100,000 for men aged 40-84 years. [10] Australia has a reported prevalence of 28%; Spain's
reported prevalence is 19% prevalence in in men older than 60 years. [3] Sporadic cases are
reported in Africa and Asia. [10]
Mortality/Morbidity
The effects of morbidity in Dupuytren contracture are generally limited to lifestyle changes.
MCP and PIP joint contractures may interfere with activities of daily living and the nodules
can be painful. Occasionally, Dupuytren contracture is associated with plantar fascial
thickening (Ledderhose disease), involvement of the penis (Peyronie disease), or involvement
of the knuckle pads (Garrod nodes). These associations tend to reflect more aggressive
disease. [52] No mortality occurs from Dupuytren contracture.
Race
Racial variation in Dupuytren disease is as follows:
Dupuytren disease is most often found in Caucasians of Northern European descent.
The disease prevalence in Asians is 3% and usually involves the palm rather than the
digits. Therefore, it is less likely to be clinically significant, and the incidence may be
underreported.
Dupuytren disease has been reported in East Africa, Zimbabwe, and Tanzania.[7]
Dupuytren disease is uncommon among Indians (< 1%), Native Americans, and
patients of Hispanic descent. [7]
Sex and age
Approximately 80% of affected individuals are male; this is consistent throughout all
countries and races. The disease onset in males tends to occur in the fifth to sixth decade.
Men tend to present a decade earlier than females. The disease course tends to be more rapid
and severe in males and increases in incidence with advancing age. [44]
Dupuytren disease is rare in children younger than 10 years. Only eight histologically proven
cases have been reported. [53]
Patient Education
Patients must have realistic expectations that surgery can relieve some disability but that it
cannot cure Dupuytren disease. Discuss all potential complications of the procedure,
including complex regional pain syndrome. In addition, intensive rehabilitation with an
occupational therapist is necessary postoperatively for an optimal outcome.
ie Danielle Mathew, DO; Chief Editor: Herbert S Diamond, MD more...

History
Obtain a thorough medical history when evaluating a patient thought to have Dupuytren
contracture. Presenting symptoms typically include the following:
Decreased range of motion
Loss of dexterity
Getting the hand "caught" when trying to place it in a pocket
Conditions possibly related to Dupuytren contracture include the following:
Diabetes mellitus
Alcohol abuse
HIV infection
Epilepsy
Trauma
 Manual labor with vibratory exposure
Cigarette smoking
Patients describe feeling a knot or thickening on the palmar surface or, less frequently, on the
digits, typically the proximal palmar aspect. Often, the thickening has been present for many
years and may be slowly progressive.
The fourth digit (ring finger) is most frequently affected, followed by the fifth digit. The
disease can be bilateral but is generally not symmetric in severity. Hand dominance is not a
factor. Nodules typically are painless, unless nerve compression or tenosynovitis is present.
Tenosynovitis can develop and lead to pain when the nodules are large. With progressive
disease, flexion deformity can develop and patients will report an inability to straighten the
fingers.
Asking about functional disabilities may elicit a history of certain tasks that the individual
can no longer perform, such as grasping objects and typing. No sensory deficits are reported
unless there is a concomitant pathology. The condition is painless in its later stages.
Physical Examination
Perform a thorough physical examination focusing on the involved extremity. A careful
physical examination often confirms the diagnosis without the need for further tests. [54]
Important points to assess include the following:
Firm nodules that may be tender to palpation: The nodules are closely adherent to the
skin; movement of the nodule with finger motion suggests an association with the
tendon and not Dupuytren contracture. See the image below.

This photo demonstrates the presence of a nodule as well as skin blanching with
extension of the affected digits.
View Media Gallery
Painless cords proximal to the nodules
Skin blanching upon active finger extension
Atrophic grooves or pits in the skin: These represent adherence to the underlying fascia.
Tender knuckle pads over the dorsal aspect of the PIPs (Garrod nodes): These occur in
44-54% of patients and suggest more aggressive disease. [3]
Plantar fascia involvement, known as Ledderhose disease (6-31%): This can indicate
more severe disease. [3]
Presence of MCP and PIP joint contractures: Objectively measure and record the degree
of flexion contracture and assess for compensatory DIP joint hyperextension or
contracture.
 Hueston table top test: If the patient is unable to lay the palm flat on a tabletop, the
findings are considered positive.
Diagnostic Considerations
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 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
outalthough the pathologic findings of a biopsy will most likely reveal a benign etiology
(eg, lipoma, inclusion cyst).
Conditions to consider in the differential diagnosis of Dupuytren contracture include the
following:
Diabetic cheiropathy
Epithelioid sarcoma
Fibroma
Giant cell tumor
Intrinsic joint disease
Lipoma
Neurofibroma
Palmar fibromatosis
Palmar tendinitis
Retinacular ganglion of the A-1 pulley
Tendon nodule of stenosing tenosynovitis
Tophi
Traumatic scars
Callus
Ganglion cyst
Prolapsed flexor tendon
Ulnar nerve palsy
Camptodactyly
Changes secondary to rheumatoid arthritis
Hyperkeratosis
Non-Dupuytren disease
Palmar ganglion
Differential Diagnoses
Clavus
Epidermal Inclusion Cyst
fibroma
Giant Cell Tumor of Tendon Sheath
Lipoma
Neurofibroma
Tendon nodule
Tophi
Approach Considerations
No routine diagnostic laboratory studies apply to this disorder. However, diabetes mellitus
has been associated with Dupuytren contracture. A fasting blood glucose level should be
obtained if diabetes mellitus is suggested by the patient's clinical history and physical
examination findings.
No routine radiographs are necessary, but ultrasonography can demonstrate thickening of the
palmar fascia, as well as the presence of a nodule. In addition, ultrasonography of a thickened
cord may be useful prior to intralesional injections so that the underlying tendon can be
identified and avoided during the injection. (See the image below.)

Ultrasound of the palm in Dupuytren disease of the fourth digit; the Dupuytren nodule and
thickening of the palmar fascia with puckering of the skin is noted. Green arrow: Flexor
tendon; Red arrow: Dupuytren nodule; Blue arrow: Thickened palmar fascia; Yellow arrow:
Puckering of the skin.
View Media Gallery
Histologic Findings
Histologic analyses of lesions resulting from Dupuytren contractures indicate physiologic
characteristics of hypertrophic scar formation, including the following: myofibroblastic
proliferation with increased water levels, an increase in type III collagen, chondroitin sulfate,
and reducible cross-linkages. In addition, surrounding tissue without clinical manifestations
reveal newly formed type III collagen, reducible cross-links, and fibroblasts with
myofibroblastic characteristics, such as intracellular microfilaments, as well as basement
lamina-like condensation. This suggests that Dupuytren disease spreads by migrating along
the altered collagen bundles and clinically silent areas are affected on a microscopic level. [55]
A histologic staging system was proposed by Luck in 1959. He described three progressive
stages, as follows [14] :
Proliferative stage: This is characterized by intense cellularity with collagen fibrils
randomly arranged and surrounded by proliferating fibroblasts
Involution stage: The collagen fibrils are organized along lines of tension; the
myofibroblast is the predominant cell and aggregates near the collagen fibrils
Residual stage: Collagen is uniformly oriented, with few to no cells and fibrosis; this
stage is analogous to that of wound healing
In all stages, a core of type I collagen is surrounded by type III collagen. Studies suggest that
the percentage of type III collagen changes with the stage of the disease. [56]
Approach Considerations
Observation
This is appropriate for patients with unchanging, painless Dupuytren disease with minimal
contracture and no functional impairment. [57] Patients with mild Dupuytren disease can be
monitored on an infrequent basis via a brief follow-up visit every 6-12 months. In addition to
accurate measurement of the progression of the contractures, the follow-up also provides an
opportunity to elicit a history of any functional deficits. Further, these visits allow assessment
and discussion of the need for surgical referral.
Physical and occupational therapy
Stretching with the application of heat and ultrasonographic waves may be helpful in the
early stages of Dupuytren contracture. The physical therapist also may recommend that the
patient wear a custom splint or brace to stretch the fingers further. Range of motion (ROM)
exercises should be performed several times a day. If the patient undergoes surgical
correction of the contracture, physical therapy often is involved following the procedure. The
postsurgical program consists of wound care, massage, passive stretching, active ROM
exercises, and splinting.
Through a course of occupational therapy, the patient may learn adaptive techniques and
begin to use assistive devices that enhance functional abilities. For example, adaptive
equipment can help a patient to open jars, despite contractures.
Corticosteroid injection
Intralesional triamcinolone acetonide (Kenalog-40) injections of 40 mg/mL have yielded
subjective improvement in the size of Dupuytren nodules in some patients.[58] Ketchum
argues that early treatment of a nodule with an intralesional steroid injection, before the
development of joint contracture (particularly PIP joint involvement), can interrupt the
inflammatory process and thus the progression of disease. [59]
However, corticosteroids are associated with a high risk of complications, including fat
atrophy and skin discoloration. [57] In addition, intralesional injection of corticosteroids can
result in tendon rupture.
Radiotherapy
Radiotherapy can be effective in slowing disease progression in the early stages of Dupuytren
contracture but was not effective in advanced disease. Radiotherapy did not reduce the rate of
surgical intervention and was associated with a high rate of adverse events. [60]
Collagenase injection
Prior to February 2010, surgical intervention was the mainstay of treatment for Dupuytren
disease despite a high rate of recurrence and complications. [61] In February 2010, the US
Food and Drug Administration (FDA) approved collagenase Clostridium histolyticum (CCH;
Xiaflex) at a dose of 0.58 mg per injection for the treatment of Dupuytren contracture in a
single digit during a 30-day treatment cycle. Injected collagenase extracted from C
histolyticum weakens and dissolves the Dupuytren cord. [62, 63]
A study by Hurst et al resulted in FDA approval of CCH for the management of Dupuytren
contracture. Hurst et al performed a prospective randomized double blind placebo controlled
trial investigating collagenase versus placebo for the treatment of Dupuytren disease, with
contractures greater than 20 in 308 patients (CORD I and CORD I Extension). Their findings
included a reduction in contractures to less than 5 in 64% of collagenase-injected patients
compared with 6.8% of patients treated with placebo. Patients with MCP involvement tended
to improve to a greater extent, as did those patients with less severe flexion contractures. [2]
Glipin et al performed a 90-day double blind placebo-controlled study with a 9-month open
label extension evaluating the efficacy of CCH in Dupuytren disease in Australia, termed
CORD II. Sixty six patients were enrolled: 45 in the treatment group and 21 in the placebo
arm. The primary end-point was reduction of the contracture to 0-5 of normal, 30 days after
the last injection. A statistically significant reduction in contracture was reported in the
treatment group, with 44% of patients meeting the primary endpoint with treatment versus
4.8% of controls. [63]
Witthaut et al reported on the efficacy and safety of CCH in 2 open label trials (JOINT I in
the United States and JOINT II in Australian and Europe). Patients with fixed-flexion
contractures of the MCP or PIP could receive up to 3 0.58-mg CCH injections per cord, with
finger extension procedures 24 hours later. Dupuytren cords affecting 879 joints in 587
patients were injected. Clinical success was achieved in 57% of joints (MCP 70% success
rate, PIP 37% success rate). The mean change in contracture was 55 for MCP joints and 25
for PIP joints. [64]
At least one adverse event was seen in 97% of patients. Most were localized to the injection
site. The most common side effects included edema if the treated extremity, bruising,
injection site pain, extremity pain, injection site swelling, and hemorrhage. Fifty five cases of
skin laceration were reported, along with 34 cases of injection-site vesicles. [64]
Peimer et al evaluated the long-term safety and efficacy of CCH in the CORDLESS study;
1080 patients were enrolled from the original 5 clinical trials (JOINT I, JOINT II, CORD I,
CORD I extension and CORD II). Patients were evaluated beginning 2 years after their first
injection and then annually for a total of 4 years. Recurrence rate was 35% (MCP, 27%; PIP,
56%). In the original studies 301 joints were partially corrected; 50% of these had a
nondurable response (MCP, 38%; PIP, 62%). [65]
Anticlostridial type I collagenase and/or anti-clostridial type II collagenase antibodies were
reported in 96% or more of patients who had received 2 or more CCH injections and 82%
who received one injection. No correlation of antibody titer to adverse events was reported.
No new or long-term serious adverse events were noted, although the recurrence rate was
noted to be lower in successfully treated joints than in partially corrected joints. [65]
Coleman et al studied the efficacy of CCH when injecting two Dupuytren cords concurrently.
The study treated 12 subjects with more than three joint contractures. During the first
treatment session, all subjects were injected with single dose of CCH (0.58 mg) into a single
cord. Thirty days later, the same patients were injected at two different cords concurrently in
the same hand. A mean reduction in joint contracture of 30 was obtained. All patients were
satisfied with the outcome, and the rate of adverse events was similar to a single injection.
This study suggested that two cords can be treated concurrently with a similar safety profile
of treating the individual cords sequentially. [66]
In October 2014, the FDA approved an expanded indication for CCH for the treatment of up
to two Dupuytren contracture joints in the same hand during a single treatment visit. [67] A
finger extension procedure can be performed at 24, 48, or 72 hours after injection.
Complications associated with CCH are common. In the Hurst et al study, an adverse event
occurred in 96% of patients, although only 3 serious reactions were reported. The most
common complications included injection site reactions, peripheral edema, bruising,
bleeding, and pain. More serious reactions included tendon rupture and complex regional
pain syndrome. To date, tendon rupture has been described in 0.3% of 1080 patients. [62]
5-Fluorouracil
5-Fluoruracil has been shown to cause a dose-dependent, selective and specific decrease in
collagen production by fibroblasts and inhibit fibroblast proliferation and myofibroblast
differentiation. [68, 69] However, TGF-beta1 gene expression and procollagen type I and III
messenger ribonucleic acid (mRNA) are not affected. After undergoing clinical trials, this
treatment may be useful as an adjuvant therapy to surgery in reducing extracellular matrix
production and recurrence of Dupuytren contracture. [16]
Imiquimod
Imiquimod is an immune modulator that downregulates TGF-beta and fibroblast growth
factor-2, which are important cytokines in the pathogenesis of Dupuytren contracture.
Immune modification of profibrotic cytokines may be an innovative way to control disease.
Imiquimod cream has been proposed as a therapy. [70] However, no reports have described
experimental use of imiquimod for Dupuytren contracture.
Botulinum toxin
This has also been proposed as an intralesional therapy for Dupuytren contracture, based on
its inhibition of Rho GTPase, which is necessary for the activation of the IL-1 inflammation
pathway. [71] As with imiquimod, no reports currently describe clinical use of botulinum toxin
for this condition.
Hyperbaric oxygen
Hyperbaric oxygen is another theoretical therapeutic option. Fibroblast and myofibroblast
production may cease if hypoxic conditions are reversed by high tissue oxygenation. [16, 72]
Surgical therapy
The goal of surgical care is to excise the diseased fascia to help prevent progression of the
disease. A patient should be referred to a hand surgeon if the MCP contracture is more than
30 or if any contracture of the PIP is present. Functional disability may be an indication for
surgery if the patient accepts the associated morbidity and understands that surgery may not
be curative. As with all elective surgeries, the patient's age, comorbid conditions, and ability
to comply with postoperative care and rehabilitation also determine whether surgery is
appropriate.
Surgical interventions consist mostly of fasciectomy. [73] Complete extension of affected
joints may be possible with earlier intervention. However, any degree of PIP contracture
carries a poor prognosis, and excising the involved fascia may not correct the joint
contracture, especially if it has been long standing. MCP involvement is more amenable to
surgical correction than PIP involvement, even in more advanced or long-standing cases.
Subsequently, surgery is recommended as soon as a PIP contracture is detected.
A study found that improvement in the PIP joint contracture has a greater correlation with
hand function at 6 and 12 months after surgery than does improvement in the MCP joint
contracture.The results of another study revealed the severity of contracture preoperatively
had a significantly negative effect on hand power, and older patients experienced less
functional benefit from selective fasciectomy. [74] In bilateral cases, the initial operation is on
the worst or dominant hand. If indicated, the other hand can be operated on 6-8 weeks after
healing of the first hand.
If a patient needs to undergo surgical fasciectomy for Dupuytren disease and also requires a
carpal tunnel release for carpal tunnel syndrome, a study recommends that both procedures be
done concomitantly, as the complication rate is not significantly increased. [75]
For more information, see Surgery for Dupuytren Contracture.
Surgery versus collagenase injection
In a study that compared CCH injection versus limited fasciectomy in 132 patients with
Dupuytren contracture, Zhou and colleagues reported no significant difference in the degree
of residual contracture of the MCP joint (13 with CCH versus 6 with surgery; P = 0.095).
With PIP joint treatment, residual contracture was significantly worse with CCH (25 versus
15 with surgery; P = 0.010). However, patients treated with CCH had more rapid recovery
of hand function and experienced fewer serious adverse events than did those treated with
fasciectomy. [76]
A prospective, single-blinded, randomized study in patients with an MCP contracture of 20
or more in a single finger found no significant differences in outcome after 1 year between
the 69 patients randomized to CCH treatment and the 71 patients randomized to needle
fasciotomy. In both groups, 90% of patients had full extension of the treated MCP joint. [77]
Consultations
Surgical intervention may be appropriate in more severe cases. Consider surgical consultation
with one of the following specialists:
Plastic surgeon
Orthopedic hand surgeon
Rehabilitation
Physical therapy
Stretching with the application of heat and ultrasonographic waves may be helpful in the
early stages of Dupuytren contracture. The physical therapist also may recommend that the
patient wear a custom splint or brace to stretch the fingers further. ROM exercises should be
performed several times a day. If the patient undergoes surgical correction of the contracture,
physical therapy often is involved following the procedure. The postsurgical program consists
of wound care, massage, passive stretching, active ROM exercises, and splinting.
Occupational therapy
Through a course of occupational therapy, the patient may learn adaptive techniques and
begin to use assistive devices that enhance functional abilities. For example, adaptive
equipment can help a patient to open jars, despite contractures.
Fasciotomy
Percutaneous needle fasciotomy/closed fasciotomy
Percutaneous needle fasciotomy (PNF) is a minimally invasive treatment that is usually
performed as an office procedure under local anesthesia. It involves multiple puncture sites
and sectioning of the Dupuytren cord using the bevel of a needle. [78, 79, 80, 81]
Needle fasciotomy may be an effective initial intervention in the treatment of Dupuytren
contracture either alone or as a first step to subsequent procedures such as repeated
fasciotomy, fasciectomy, or CCH injection. [82]
In a study of 211 older patients (average age 65 y), investigators found a single digital nerve
injury, no infections, and no tendon injuries with needle aponeurotomy, although damage to
the neurovascular bundle is a concern. Recurrence rates (58%) and disease activity (69%)
were high at 3-year follow-up. [83]
Foucher et al believed this technique was ideal for the elderly patient with a bowing cord and
a predominant MCP joint contracture. Needle fasciotomy may also facilitate hygiene in a
debilitated elderly patient. Limitations of fasciotomy in treating PIP joint contracture were
noted. [83]
Contraindications to PNF include infiltrating disease; rapid recurrence in a young patient;
inaccessible, multiple cords; chronic digital disease; and postsurgical recurrence in the
digits. [83]
Open fasciotomy
An open fasciotomy is sometimes used to manage more severe cases of Dupuytren
contracture, and is more effective in the long-term when compared with needle
aponeurotomy. Open fasciotomy is still an outpatient procedure performed under local
anesthesia. An incision is made over the diseased cord and direct visualization of the cord and
neurovascular structures is possible. The offending cord is divided at a point immediately
underlying the skin incision.
Fasciotomy is usually most successful for MCP flexion contracture. The recovery is rapid,
but the recurrence rate is high. Open fasciotomy is usually reserved for patients who cannot
tolerate a more extensive procedure.
Segmental aponeurectomy/fasciotomy
Segmental aponeurectomy of Moermans is a procedure that is intermediate between simple
fasciotomy and limited fasciectomy. Segments (1 cm in length) of fascia are excised through
C-shaped incisions. [84, 85]
Fasciectomy
Regional (or selective) fasciectomy
This involves excising only the fascia that is grossly affected (eg. in the palm, pretendinous
cords and involved natatory ligaments; in the fingers, only those structures that are visibly
affected). Although the disease process clearly extends into clinically normal palmar fascia,
this approach has proven successful in correcting MCP joint contractures and some PIP
contractures and carries an acceptably low morbidity rate.
Areas not treated may develop disease. This method is commonly used to treat primary and
recurrent disease. Skin incisions may be transverse, longitudinal, or diagonal/zigzag (eg, Z-
plasty, Y-V-plasty, Bruner-type zigzag incision). Local advancement flaps, including an
ulnar-based skin flap [86] and palmar inter-metacarpal flap [87] have been described.
In a study of selective fasciectomy, Hueston concluded that this procedure does not prevent
recurrence of Dupuytren disease but does allow correction of deformity with more rapid
recovery of hand function. [88]
Extensive or radical fasciectomy
This involves excision of the entire palmar fascia, including tissue that appears grossly
healthy (in an effort to prevent recurrence). Although this procedure is not commonly
performed today, it results in a relatively low recurrence rate of about 11%. [89] It does have
an increased risk of morbidity postoperatively, including hematoma in 14% of cases and
nerve irritation or damage in 6%. Patients are also prone to prolonged postoperative edema
and stiffness. One study concluded that total aponeurectomy was most appropriate for stage 2
disease. [89]
Dermofasciectomy
This removes the diseased fascia and the overlying skin. The wound is then resurfaced and a
full thickness skin graft is applied. Recurrence rates are low, being similar to those of
extensive fasciectomy. [89] Because of the radical nature of this procedure, it is usually
reserved for patients with recurrent or severe disease.
In dermofasciectomy, two incisions are made, one from the distal interphalangeal joint of the
affected digit to the distal palmar flexion crease, and a transverse palmar incision, to form
an L shape. A selective fasciectomy is performed, with partial closure of the incision site. A
full-thickness skin graft is harvested from the hypothenar eminence during this surgery. A
portion of the palm is left open, and an extension splint is applied. After 4 days, the splint is
removed, the wound is cleaned, and the skin graft is applied to the palm. The palm is splinted
again for 1 week.
General recommendations for surgical intervention
A fasciotomy or regional fasciectomy is usually sufficient to establish normal function in the
MCP joint. The procedure of choice for PIP joint involvement is either dermofasciectomy or
extensive fasciectomy.
Amputation may be recommended if digital contracture is greater than 90 or if vascular
compromise has developed. Some patients may prefer amputation to the postoperative care
required for fascial surgery.
Surgical Considerations
The surgeon and the patient may choose general or regional anesthesia during the procedure.
Loupe magnification is used to aid visualization and detection of the delicate structures,
specifically the neurovascular bundle.
A pneumatic tourniquet is typically used on the operative extremity to control blood loss and
assist with visualization.
Incisions vary and may be transverse, zigzag, or longitudinal, depending on the region
involved. The skin is separated from the underlying diseased palmar fascia and all
neurovascular bundles that may be jeopardized during dissection are identified. The
neurovascular bundles are often displaced or distorted by the contraction of the components
of the palmar fascia.
After each neurovascular bundle has been identified and dissected away from the diseased
palmar fascia, the diseased fascia is excised. The MCP joint is often fully corrected with this
maneuver. PIP joints may have residual flexion at this stage and may require release of the
flexor tendon sheath, as it can become shortened with chronic contracture.
The skin is closed with running or interrupted absorbable or non-absorbable suture material.
A modified skin closure with Z-plasty or V-Y advancement can provide additional length
without undue tension. If skin grafting is necessary to close the wound, use a full-thickness
graft to minimize wound contracture during healing. Postoperatively, bandage the hand and
place it in a splint.
Wound Closure
Skin overlying the contracture is closed either primarily (using skin grafts) or by secondary
intention.
Open-palm technique
The open-palm technique (McCash technique) involves a transverse skin incision and
division of the aponeurosis; healing is by secondary intention.
Jacobsen flap
A modification of the McCash technique, called the Jacobsen flap, uses the L -shaped
incision of dermofasciectomy, but healing is by secondary intention. [90]
Synthesis technique
The synthesis technique is a method of wound closure that incorporates the advantages of
tissue rearrangement, the open-palm technique, and full-thickness skin grafting. One study
showed a decreased healing time and recurrence rate using the synthesis technique versus the
traditional open-palm technique. [91, 92, 93]
Postoperative Care
Routine postoperative care is essential for an optimal outcome. Immediately following the
procedure, a splint should be applied dorsally to avoid excess pressure on the incision site.
The hand should be placed in extension with flexion of the MCPs and extension of the PIPs.
Initially, the splint is worn continuously, with removal only for dressing changes and
exercise. [94] Patients who undergo PIP surgery should wear the brace for 6 weeks on a
continual basis and may require 3 months of bracing to minimize scar contractures.
The overall goal of splinting is to provide a prolonged stretch to the tissues and prevent
flexion contractures. Splinting is modified over the next 8-10 weeks to accommodate range of
motion, with the splint worn primarily at night.
A specific exercise regimen with an occupational therapist should be instituted with range-of-
motion exercises 1 week postoperatively. A rehabilitation program is critical for successful
management of these patients; it helps to reduce swelling, improve wound healing, and
restore finger mobility and function. [95]
Rehabilitation following surgery is a gradual process of increasing activity and decreasing
splinting to achieve increased range of motion. According to one study, therapy that avoids
applied mechanical tension in the early postoperative phase results in fewer complications,
with no digital motion lost to extension. [96]
The occupational therapist regularly records the patients degree of extension to assist in
monitoring the patients progress. Maximal results are evident 6 weeks postoperatively.
Return to normal activity is expected in 2-3 months.