Given the progressive nature of Dupuytren’s contractures, this author provides a closer look at the diagnosis of the condition, reviews the literature on possible risk factors and offers insights on treatment modalities.

Hand contractures are a devastating disability that can significantly affect the activities of daily living as well as one’s quality of life. Dupuytren’s disease is one of many causes of hand contractures. With proper diagnosis and timely treatment, patients can maintain function and quality of life.

Dupuytren’s is a progressive thickening of the palmar fascia, which develops into cords that eventually cause contractures of the fingers at the proximal interphalangeal (PIP) and/or the metacarpophalangeal joints (MCP).

One would diagnose Dupuytren’s contractures via physical examination and patient history. In these cases, the clinician can palpate palmar nodules and/or cords, and these cords prevent extension of the PIP and MCP joints. Often, there is no history of trauma.

When it comes to the earliest occurrence of a Dupuytren’s contracture, a patient may present with a small nodule near the distal palmar crease that is directly in line with one of the fingers. This nodule can feel like, and be mistaken for, a callus. However, as the disease progresses, it may cause the skin to pucker. The first nodule can also present in the finger over the proximal phalanx. However, unlike the nodule at the distal palmar crease, this nodule can lie just to the side of the flexor tendon sheath.

As the disease progresses, the nodules grow into cords and start to cause contractures. The rate of contracture varies greatly. Unfortunately, these patients, on average, do not seek out a clinician until 10 years after the initial signs and symptoms.¹ The office visit usually occurs when the contracture starts interfering with a patient’s daily activities. Some patients describe a degree of discomfort with direct pressure early on in the disease despite the fact the cords are generally not painful.

The palmar cords cause contraction at the MCP joints and the finger cords cause contraction at the PIP joints. These cords will not allow the fingers to extend but the patient is still able to flex his or her hand into a full normal fist. However, there is no ulnar deviation to the contracture as one would see with rheumatoid arthritis.

Here one can see the skin changes associated with Dupuytren’s contractures. Note the nodule at the distal palmar crease, the skin puckering and the appearance of a callous.

Dupuytren’s disease is usually bilateral with symptoms in one hand being more severe. This severity has no correlation with the patient’s hand dominance. The small finger is most often affected (70 percent of cases of Dupuytren’s contractures), followed by the ring finger and then the middle finger respectively.¹ The thumb and index finger are least likely to be affected but cords to the index finger-to-thumb web space can develop.

Here is a Dupuytren’s nodule in the small finger.

Pertinent Pointers On The Differential Diagnosis
Dupuytren’s contractures develop on the glabrous (non-hair forming) skin of the hand. Other associated disorders also develop fibromatosis on glabrous skin. These ectopic manifestations include Ledderhose's disease, Peyronie’s disease and Garrod’s knuckle pads. Ledderhose’s disease is plantar fibromatosis, which develops on the soles of the feet. Peyronie’s disease is fibromatosis that affects the penis. If a patient presents to you with any of these associated diseases, he or she may have knuckle pads over the dorsum of the PIP joints. These are also known as Garrod’s knuckle pads.

If a younger patient has rapidly developing, bilateral Dupuytren’s contractures, along with a strong family history and one or more of the above associated fibromatosis disorders, he or she may have Dupuytren’s diatheses. Dupuytren’s diathesis is a severe and rapidly progressive form of the disease that clinicians may see among younger individuals, and is associated with a poorer outcome.¹

In these photos, one can see the development of a palmar cord. As the disease progresses, the skin puckering decreases and the cord and contracture increase.

There are multiple conditions that may appear to be the early onset of Dupuytren’s contractures. Some of these conditions are masses such as ganglion cysts, inclusion cysts, giant cell tumors or epithelioid sarcomas. Some cause contractures such as intrinsic contractures, stenosing tenosynovitis (trigger finger), rheumatoid arthritis or camptodactyly. Accordingly, clinicians should include these conditions in the differential diagnosis. However, these conditions fail to have the clinical progression of the contracture associated with the palpable cord.

Do Genetic Factors Play A Role With Dupuytren’s Disease?
Dupuytren’s contractures occur predominantly among Caucasian males of northern European descent over the age of 60 years. Men are seven to 15 times more likely to be diagnosed with Dupuytren’s and have an earlier peak onset in their 70s as opposed to a peak onset in the 80s for women. However, by the time patients reach their 90s, the prevalence is equal.^1,2

The Celtic population shows an incidence of more than 25 percent. The term “Viking disease” has been coined because of the high occurrence in Northern Scotland, Iceland, Norway and Australia.3 This prevalence in a particular ethnic group and the continued familial clustering that researchers have observed continues to support the evidence of an inherited autosomal dominant gene with variable penetration.⁴

This suggests that gene mutation may have occurred in the Celtic or Germanic tribes in northern Europe between 1200 and 200 B.C.⁵ However, one of the complexities is that Dupuytren’s disease only has a 10 percent association of a positive family history.

Therefore, we must look at other factors that may influence the development of Dupuytren’s contractures.

The normal palmar fascial structure can be affected by Dupuytren’s contractures. (Photo reprinted with permission from Trumble TE. Principles of Hand Surgery and Therapy. 1st edition, Philadelphia: W.B. Saunders Company, page 456.)

Are Dupuytren’s Contractures More Common In Smokers And Alcoholics?
Alcohol use is commonly associated with Dupuytren’s disease. In the 1950s, a study found that the prevalence of Dupuytren’s was 66 percent in alcoholics with cirrhosis of the liver and 27 percent in alcoholics without cirrhosis. Subsequent studies from the 1980s confirmed the prevalence of Dupuytren’s disease in alcoholics but did not find an increase in those with cirrhosis. The cause and effect relationship between alcohol use and Dupuytren’s disease is unclear as is the mechanism of action.³

Smoking has a more modest association than alcohol but still represents a strong risk factor for the development of Dupuytren’s contractures.6 The fibrosis of the palmar fascia may develop from local hypoxia, the production of xanthine oxidase and the development of free radicals. This theory was tested when allopurinol was used to decrease xanthine oxidase without any benefit. There should also be a decrease of Dupuytren’s in patients treated with allopurinol for gout but this is not the case.^3,7

Diabetes mellitus is prevalent in approximately 20 percent of patients with Dupuytren’s contractures. Patients with type I diabetes tend to be affected at a younger age. This may be due to local hypoxia as one would see with smokers or the possibility that these two diseases are inherited together. There is no correlation between glycemic control and the severity of the contractures. Cases of Dupuytren’s contractures in patients with diabetes tend to be less severe and accordingly do not require surgical treatment.³
Recently, researchers discovered that 36 percent of severe HIV patients have Dupuytren’s contractures. This finding suggested the contribution of free radicals in the development of the disease.³

In 1941, Dupuytren’s contractures occurred in 50 percent of male epileptic patients and 25 percent of female epileptic patients using anticonvulsants due to a propensity to develop chronic fibrotic conditions. This finding was based on patients in specialist epilepsy centers as opposed to the general population afflicted with epilepsy.³ More recent studies have found little conclusive evidence linking Dupuytren’s contractures and epilepsy.⁸

Here one can see a positive table-top test for a patient’s left hand. The patient has significant flexion contractures at her PIP joints, 25 degrees at her index finger. She is a candidate for treatment.

What You Should Know About Other Possible Risk Factors
Many other risk factors are known to be associated with the onset of Dupuytren’s contractures. These risk factors include: socioeconomic status, body mass index (BMI) and repetitive use or trauma. All of the above risk factors have studies to both support and refute their findings.

One such study looked at alcohol use and smoking and found that a low socioeconomic status and a low BMI contributed as co-factors to an higher incidence of Dupuytren’s.⁶ The cause and effect cannot be verified because they were only looking at individuals that smoke and/or drink alcohol.

The association between repetitive use or trauma and the development of Dupuytren’s was even described by the Baron Guillaume Dupuytren himself when he discovered that his driver developed Dupuytren’s, possibly from holding horse reins.³ A more recent study from 2004 found a higher prevalence of Dupuytren’s contractures in “committed male rock climbers.”⁹

There have been several studies that find no correlation between repetitive use and Dupuytren’s disease. For example, a study from the 1950s looked at 1,000 steel workers, miners and clerks, and found no difference in the development of Dupuyten’s contractures based on how they used their hands.³
There is one disease that is associated with a decreased incidence of Dupuytren’s contractures. Rheuma-toid arthritis is the only condition that has a lower incidence of Dupuytren’s disease. This could be due to the use of rheumatologic medications.³

There is evolving evidence of a non-Dupuytren’s disease of the palmar fascia in which there is a non-progressive thickening of the palmar fascia. There is no family history, no gender predilection and no ectopic manifestation. There is unilateral ethnic diversity. The development of the cord often follows trauma or unrelated hand surgery. Patients develop a palmar cord and a slight contracture at the MCP joint may develop, but it does not progress or affect the PIP joint.¹⁰

In the above photo, one can see a positive table-top test of another patient’s left hand. Note the significant flexion contractures at her PIP joints, 55 degrees at her small finger. She is a candidate for treatment.

While many studies have assessed possible risk factors for the development of Dupuytren’s contractures, there has been a lack of conclusive findings. Race, genetic predisposition, age, male gender, a history of diabetes mellitus and use of alcohol and cigarettes are all associated with an increased risk of developing Dupuytren’s contractures.

In regard to epilepsy, HIV, socioeconomic status, body mass index (BMI) and repetitive use or trauma, evidence is mixed on what roles they might play in the development of Dupuytren’s contractures.

Understanding The Pathophysiology Of Dupuytren’s Disease
Cellular changes that occur with the development of Dupuytren’s disease are similar to the changes one would see in wound healing. There is a proliferation of type III collagen and myofibroblasts, which are a contractile of cells. While this proliferation facilitates wound healing when injured, it causes contractures of the palmar fascia among people with Dupuytren’s disease. There is a clear understanding of how this takes place. Why it happens to the non-injured palmar fascia still eludes clinicians.^2,11

The palmar fascia is a strong aponeurosis that lies just below and is attached to the dermis with small vertical fibers. This attachment holds the palmar skin in place and assists with grasping objects. The palmar fascia contains several structures that can be affected by Dupuytren’s contractures.

These structures, from proximal to distal, start with the pretendinous band, which connects to the spiral band. This also connects to the natatory ligament and the lateral digital sheet. Grayson’s ligament is palmar to the lateral digital sheet and Cleland’s ligament is dorsal to the lateral digital sheet.

Here is a view of the aforementioned patient’s right hand. There was a negative table-top test for this hand. She does have a slight palpable cord in the palm of this hand but it is not ready for treatment.

These three structures form a channel for the neurovascular bundle. When Dupuytren’s disease affects these normal structures, the pretendinous band becomes the pretendinous cord, the natatory ligament becomes the natatory cord, and the lateral digital sheet becomes the lateral digital cord. The distal part of the pretendinous band, the spiral band, the lateral digital sheet and Grayson’s ligament become the spiral cord. Since the lateral digital sheet and Grayson’s ligament form part of the channel for the neurovascular bundle, the spiral cord can displace it central and palmar. This greatly increases the risk of damage to the nerves and arteries during treatment for Dupuytren’s contractures.^4,11

Essential Considerations In Treatment
There are a number of factors to consider when weighing the decision of whether to treat Dupuytren’s contractures. Clinicians should consider the severity of the contracture, the rate at which the disease is progressing, the type of treatment, your patient’s age, comorbid conditions and his or her understanding and willingness to comply with the sometimes lengthy, post-treatment rehabilitation.

If one initiates treatment too soon, the disease could accelerate and produce a greater morbidity. If treatment is delayed, the contracture may become so severe that treatment may not be fully successful. It has become generally acceptable to initiate surgical treatment when a patient’s MCP joint has a 30-degree flexion or more, and/or there is a contracture at the PIP joint.

One can determine this simply by using the Hueston tabletop test. Have the patient place his or her hand palm down on a table. If he or she is unable to lay the hand flat on the table and if you can see daylight between the hand and the table, one should consider treatment.^4,11

Complete surgical excision of the Dupuytren’s cord (fasciectomy) is the standard treatment. In regard to the surgical approach, it is made through a palmar incision in either a zigzag pattern or a longitudinal incision broken up by Z-plasties. With chronic finger contractures, the skin shrinks. When the cord is removed and the finger is allowed to straighten, there is not enough skin to cover the wound. Accordingly, the surgeon would make the incision in such a way that it effectively lengthens the skin for adequate coverage after one has excised the cord.

In surgical correction, it is important to utilize a zigzag incision to lengthen the skin in order to facilitate postoperative coverage of the wound and to prevent scar contractures.

Some surgeons leave the incisions open and allow the wound to heal under secondary intention. Other surgeons use full-thickness skin grafts for coverage if there is not enough skin to cover the wound. The process of complete excision of the cord can be tedious and time consuming. Using a tourniquet, loop magnification and microvascular instruments, the surgeon must tease the cord off of the neurovascular bundles without damaging them.^1,2

After the surgical fasciectomy, the patient immediately starts therapy. One can consider a variety of splints to hold the fingers straight and allow full range of motion (ROM). These patients should see a certified hand therapist two to three times per week for about six weeks. It can take up to, and possibly more than, three months before the patients can fully return to their normal activities.²

Is The Percutaneous Needle Fasciotomy The Best Surgical Option?
Numerous other treatment options have been and are still being developed to treat Dupuytren’s contractures.
In 1777, Henry Cline first used the fasciotomy. The fasciotomy fell out of favor in the 1840s when general anesthesia was introduced. There was a resurgence of the procedure in the 1980s due to the work of the French rheumatologist Jean-Luc Lermusiaux, MD.

Here one can see a surgical view of Dupuytren’s pretendinous cord versus a normal fascia.

The percutaneous needle fasciotomy is gaining popularity in this country. The percutaneous needle fasciotomy is an office procedure in which clinicians utilize a 25-gauge needle to transect the palmar cord. With this procedure, clinicians can only use a small amount of local anesthesia to avoid blocking the nerve. This enables surgeons to assess if they are getting too close to the nerve and causing damage. After the procedure, the patient is not typically sent to the certified hand therapist but is encouraged to start using their fingers.

The percutaneous needle fasciotomy has quickly gained favor with patients because it is less invasive and has a shorter recovery time. While the procedure does correct the contracture, it has a much higher and more rapid rate of recurrence. The contracture can start to recur in as little as nine months.
That said, the percutaneous needle fasciotomy is a useful tool for treating Dupuytren’s contractures in elderly patients who cannot tolerate surgery and for whom recurrence is less of a factor. However, when it comes to patients who need to return to and maintain full function, clinicians should reserve this option only when there is a need to delay the inevitable surgical fasciectomy.¹²

A Closer Look At Non-Surgical Modalities
Clinicians have utilized several non-surgical treatments to treat Dupuytren’s contractures. These options include splinting, radiation, dimethyl sulfoxide, vitamin E, anti-gout medication, ultrasonic therapy and interferon-g. Unfortunately, most, if not all of these alternatives have failed to adequately treat the contractures. A new non-surgical treatment uses clostridial collagenase injections. One would utilize the collagenase to break up the cords and allow finger extension.¹³ While researchers have shown encouraging early results, the modality is still under investigation.

None of these treatments offer a cure. The cords, and potentially the contractures, will eventually return. The goal of treatment is to delay the return of the contracture and help maintain function as long as possible.

In Conclusion
Once you have seen a few cases of Dupuytren’s contracture, it is not difficult to identify. The disease is rarely associated with any history of trauma and generally has a slow, progressive development over many years.

When it comes to Dupuytren’s contractures, clinicians will see a classic palmar cord that causes contractures of the MCP or PIP joint, most often in the small and ring fingers. This results in the patient being unable to extend his or her fingers, but he or she is still able to make a full normal fist.
As noted above, Dupuytren’s contractures are more common among men in their 60s who have a northern European ethnic background. Dupuytren’s disease has a genetic component and is associated with alcoholism, smoking and diabetes mellitus.

Patients may have an early onset and severe form of the disease, which is referred to as Dupuytren’s diathesis. In these cases, a patient may have a cord to the sole of the feet or the penis or have knuckle pads over the PIP joints. With proper diagnosis and treatment, patients can maintain function for many years.