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Dupuytren Contracture

Contracture Dupuytren's

Dupuytren contracture is a progressive fibroproliferative disease of the palmar fascia characterized by flexion deformity of the hand. 


Presentation

The earliest features of Dupuytren Disease are pitting and thickening in the palmar skin. The thickening is progressive and is likely present for years. However, typically, the initial presenting sign is a nodule in the palm, particularly near the fourth or fifth digits. These are the most frequently affected digits. The nodule is observed as a firm mass fixed to skin and fascia. While pain and discomfort may be present, they subside. The next stage is characterized by a cord formation. Gradually over months or years, contracture of the cord occurs and draws the MIP and PIP joints inwards. Hence, patients will manifest flexion deformity and experience difficulty with straightening of their fingers.

This disease can manifest bilaterally although one hand may exhibit more severity than the other. All digits can be affected with preference for the fourth and fifth [15]. Approximately 7% to 10% of patients exhibit fibrous thickening of the PIP joints and fibromatosis of the penis. In rare cases, plantar fibromatosis occurs.

While nodules do not produce pain, nerve compression and tenosynovitis cause tenderness. The latter condition occurs with larger nodules. On physical exam, the nodules may be tender to palpation. Additionally, they are observed as adherent to the skin. If the nodule moves along with finger motion, this is indicative of tendon involvement and not Dupuytren contracture.

It is important to differentiate the flexion deformity in Dupuytren contracture from the other types associated with diabetes mellitus, systemic sclerosis, and chronic reflex sympathetic dystrophy.

Patients frequently complain about contractures and feeling a knot on their palmar surface or digits. They typically feel embarrassed about their hand appearance. Furthermore, individuals with this deformity experience challenges with manual labor or daily activities.

Fishing
  • FISH analysis performed in 11 cases (centromeric probe specific for chromosome 8) failed to show the presence of a cell population with 8.[ncbi.nlm.nih.gov]
  • The fish technique. Acta Orthop Belg. 2006, 72: 678 – 82. Google Scholar Medline Smith, P, Breed, C. Central slip attenuation in Dupuytren’s contracture: a cause of persistent flexion of the proximal interphalangeal joint.[doi.org]
Hand Pain
  • Every week people report they move their fingers better, flatten their palm farther, have less hand pain and enjoy greater ease doing the important activities they lost (washing, dressing, working, etc.).[dupuytrens-contracture.com]
Skin Fissure
  • Complications include digital nerve and artery injury, flexor tendon injury, skin fissures and wound healing complications, hematoma, infection, flare reaction/complex regional pain syndrome, and recurrence.[ncbi.nlm.nih.gov]
Hyperalgesia
  • Other postoperative pain Local hyperalgesia, possibly due to digital nerve injury and neuroma formation, can be problematic. [57] Recurrence Recurrence (ie, Dupuytren tissue forming in the area of resection) and recurrence of flexion deformity with disease[emedicine.com]

Workup

A thorough history including that of family and ancestry is a component of the work up. This is followed by a detailed physical exam observing the clinical features. Suspicion for this disease is raised in patients with risk factors (male gender, smoking, diabetes, alcoholism, and Northern European descent) and the characteristic findings such as flexion deformity, palmar thickening, and nodules.

There are no laboratory tests specifically for Dupuytren contracture but studies may be warranted in patients suspected to have diabetes mellitus (through history and physical exam), in which fasting blood glucose level is indicated.

As for imaging, ultrasonography may be helpful in revealing the thickening in the palmar fascia and identifying a nodule.

Histology can confirm diagnosis by observing myofibroblastic proliferation, type III collagen, chondroitin sulfates and other characteristics as well.

Microalbuminuria
  • The disease seems to occur at a younger age and tends to be more severe in those with type 1 diabetes. [44] Patients with diabetes mellitus and Dupuytren disease have a 4-fold increased risk of developing microalbuminuria than do persons with diabetes[emedicine.com]
Staphylococcus Aureus
  • In five, all with a skin graft, Staphylococcus aureus was grown, but none required debridement and in all the infection settled uneventfully. One patient had necrosis of the tip of the Z-plasty which was allowed to heal naturally.[doi.org]
HLA-DR3
  • HLA-DRB1*15 and HLA-DR3 have been identified in numerous patients, suggesting an immunologic influence.[emedicine.com]

Treatment

There are various treatments available for Dupuytren contracture that depend on the severity and the specific joints and digits involved.

Surgical interventions

For patients with profound contracture at the PIP joints and inability to straighten their fingers, surgery is an option. Surgical intervention serves to hinder progression of the contracture by removal of the abnormal fascia. Fasciectomy is the most common surgical intervention [16] [17] [18]Surgical candidates are patients exhibiting: 1) any degree of PIP contracture 2) greater than 30 degrees of MCP contracture, or 3) disability. Better outcomes are associated with earlier intervention. If the contracture has well progressed, removal of the defected fascia does not usually result in improvement. Additionally, there are morbidities associated with the procedure as well as a risk of recurrence if the disease continues to progress. 

Open palmar and digital fasciectomy is the surgery of choice for patients with multidigit involvement. The procedure can be challenging due to the close proximity of nerves, blood vessels and tendons.

Another procedure, percutaneous needle fasciotomy, can be performed as initial therapy or as a bridge to further fasciotomies, fasciectomy or collagenase clostridium histolyticum injection [19]. This is a minimally invasive outpatient procedure performed under local anesthesia. In certain cases, dynamic external fixation of PIP joint may be an option. 

Drug and other therapies

For patients experiencing pain without contractures, intralesional corticosteroid injections may be helpful. Note that the self limiting pain does not usually require treatment. Caution should be exercised with corticosteroids due to side effects such as atrophy [20] and tendon rupture.

Collagenase Clostridium histolyticum (Xiaflex) was approved for one digit involvement contracture therapy in a monthly cycle. Injection of this may be helpful in patients with MCP joint contractures

Radiotherapy is another option for patients with early disease. Furthermore, intranodal triamcinolone acetonide resulted in moderate improvement [21] and could be useful. 

Targeting the cellular level, 5- Fluoracil can reduce fibroblast proliferation and fibroblast differentiation into myofibroblast [22] [23]. Further, there is the option of imiquimod treatment. This promising treatment is an immune response modulator of the primary cytokines involved in Dupuytren contracture development. 

Patients do not require close follow up. Once or twice a year is sufficient. Physical and occupational therapy is advised in addition to special exercises involving range of motion activities. Braces and splints may show some benefit.

Also, clinicians should address modifiable risk factors such as smoking, alcoholism, and diabetes as these contribute to other medical conditions.

Prognosis

There is no mortality that stems from this disease. Dupuytren contracture affects quality of life and hinders daily activities. Contractures of the MCP and PIP joints can be painful, uncomfortable, and limiting. But the pain is self limiting. 

Severity is associated to thickening of the plantar fascia, involvement of the knuckles, or fibrosis of the penis [14].

Patients with earlier onset and positive family history may experience post surgery recurrence.

Etiology

The etiology of Dupuytren contracture is not established. However, a genetic predisposition tends to exit. It is likely inherited in an autosomal dominant with variable penetrance pattern. 

This disorder is more common in males as they are three times as likely to manifest the disease [1] [2]. It is suggested that the male preference is correlated to androgen receptors in the Dupuytren fascia [3].

There is likely a multifactorial cause as there are potential risk factors for Dupuytren contracture such as diabetes mellitus, hyperlipidemia, alcoholism, and smoking. Hand related risk factors include previous trauma or injury and physical occupation with vibration exposure. Other associated risk factors include Peyronie disease and complex regional pain syndrome [4].

A protective factor is Rheumatoid arthritis.

Epidemiology

The disease is found predominantly in elderly men with Northern European ancestry [5]. There is a predisposition for men as they account for 80% of all affected individuals. Additionally, the age of onset in men is 55 years while it is a decade later in women [6]. Furthermore, the clinical picture is more severe in males [7].

The prevalence in the United States is 4% which is associated with Northern European immigration [5]. It is highly prevalent in North Europe exhibiting a range of 4% to 39%. Specifically, the disease affects 30% of males above 60 years old in Norwegian countries. In British males ranging from 40 to 84 years old, the incidence is 34.4 per 100,000. In Spaniard males above 60, the prevalence is 19% [8]. The Australian prevalence is 28%. In Asians, the prevalence is 3% which usually affects palms as opposed to digits. 

Sex distribution
Age distribution

Pathophysiology

It is believed that genetically susceptible individuals with triggers from underlying risk factors such as smoking, diabetes mellitus, or alcoholism can develop microvascular ischemia [9]. This is followed by two biochemical mechanisms in which hypoxanthine and xanthine oxidase are formed in the palmar fascia. A series of reactions occurs in which xanthine catalyzes the conversion of hypoxanthine to xanthine, uric acid as well as free radical byproducts. The latter cause proliferation of fibroblasts. Additionally the free radicals produce cytokines such as Interleukin 1 (IL-1), which is elevated in Dupuytren Disease. IL-1 stimulates production of various proteins such as fibroblast growth factor, epidermal-derived growth factor, platelet-derived growth factor, and transforming growth factor beta (TGF-beta) [3] [10]. The abundance of these cytokines and growth factors promote the proliferation of fibroblasts, which transform into myofibroblasts [11]. The latter is the predominant cell type in this disease.

The cause of Dupuytren contracture is not well elucidated but it has been established that the disease process involves connective tissue. Dupuytren contracture is considered a fibroproliferative disorder. In the early stages, fibroblast proliferation is one of the key observations [12]. Clinically, this is observed as the nodule. Furthermore, the early stages feature characteristics similar to those of malignancies (for example fibrosarcoma) [13]. 

As Dupuytren Disease progresses into later stages, proliferation subsides as the abnormal connective tissue organizes and forms the cord, which is a clinical feature of this phase.

Prevention

There is no prevention for Dupuytren contracture as it is a genetic disorder that likely occurs with risk factor interplay. However, some of these risk factors such as smoking, chronic alcohol use, hyperlipidemia, and diabetes mellitus are modifiable. Cessation of the first two and cholesterol and glycemic control may help delay onset or progression. Furthermore, implementing these modifications will make a difference in the overall health of the patient. 

Of note, earlier medical attention is associated with better outcomes. Therefore, if patient notices thickening of the palms, or nodules, it is best to seek care sooner. 

Summary

Dupuytren contracture is a progressive fibroblastic disease that involves the palmar fascia. It mostly affects older men of Northern European descent. This disease is likely autosomal dominant with variable penetrance. The etiology is not well established but a multifactorial cause contributes to the pathogenesis. This progressive disease involves excessive fibrosis and thickening of the palmar fascia. Furthermore, it gradually results in marked digital contracture especially in the metacarpophalangeal (MCP) joints or the proximal interphalangeal (PIP) joints of the fourth and fifth digits. 

The early stages manifest as a painless nodule and the later stages as a cord. Over times, contracture of the cord occurs, leading to deformity. The appearance is that of a claw like hand, which can cause limitation in the range of motion as well restriction of daily activities. 

Surgical intervention and treatment options exist. The former aims to excise the diseased fascia. However, it can result in recurrences and morbidity. Additionally, collagenase, radiotherapy, and imiquimod are available depending on the presentation and severity of the clinical picture. Physical and occupational therapy are also key in improving functional use of the hand. 

Patient Information

Dupuytren contracture is a disorder in which the fibrous tissue inside the palms become tight. This eventually causes the curling of the fingers and the patient may have difficulty in straightening them. In fact, the hand resembles a claw.

It is likely hereditary. It mostly affects older men of Northern European descent. While the cause is not known, there are risk factors such as diabetes, smoking, alcoholism, and epilepsy.
The first symptom is a nodule that presents on the fourth or fifth finger (although it can affect any finger). Initially, it causes some pain or discomfort but that subsides. Over time, the hand becomes claw like as described above.

Surgery may be needed to remove the thick tissue although this is tricky because blood vessels and nerves lie close together in the hand. There are different types of procedures. However, late disease may be harder to reverse. 

There are various treatment methods such as steroid injections for pain, if present. Also collagenase, a substance that can break down the bad tissue, can be beneficial. There are other options as well that the doctor can offer a patient, depending on the severity of the disease, 

This disorder causes limitations in daily activities such as washing dishes and doing laundry. Patients also experience difficulty at work especially with manual labor. Therefore, occupational and physical therapy are very important as specialists in these fields help the patients modify use of their hands. Patients are also taught to perform special exercises to practice. 

References

Article

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  2. Anthony SG, Lozano-Calderon SA, Simmons BP, Jupiter JB. Gender ratio of Dupuytren's disease in the modern U.S. population. Hand. 2008; 3(2):87-90.
  3. Al-Qattan MM. Factors in the pathogenesis of Dupuytren's contracture. Journal of Hand Surgery. 2006; 31(9):1527-34.
  4. Hindocha S, McGrouther DA, Bayat A. Epidemiological evaluation of Dupuytren's disease incidence and prevalence rates in relation to etiology. Hand. 2009; 4(3):256-69.
  5. Fitzgerald AM, Kirkpatrick JJ, Naylor IL. Dupuytren's disease. The way forward?. Journal of Hand Surgery. 1999; 24(4):395-9.
  6. Ross DC. Epidemiology of Dupuytren's disease. Hand Clinics. 1999; 15(1):53-62,
  7. Hurst LC, Badalamente MA, Hentz VR, et al. Injectable collagenase clostridium histolyticum for Dupuytren's contracture. New England Journal of Medicine. 2009; 361(10):968-79.
  8. Wheeless C. Wheeless' Textbook of Orthopedics: Dupuytren's Contracture. Ortho Preferred. Available at http://wheelessonline.com/ortho/dupuytrens_contracture. Accessed: September 22 2010.
  9. Gudmundsson KG, Arngrímsson R, Sigfússon N, Björnsson A, Jónsson T. Epidemiology of Dupuytren's disease: clinical, serological, and social assessment. The Reykjavik Study. Journal of Clinical Epidemiology. 2000; 53(3):291-6.
  10. Kopp J, Seyhan H, Müller B, et al. N-acetyl-L-cysteine abrogates fibrogenic properties of fibroblasts isolated from Dupuytren's disease by blunting TGF-beta signalling. Journal of Cellular and Molecular Medicine. 2006; 10(1):157-65.
  11. Bansal V, Naidu SH. Dupuytren's disease. Current Opinion in Orthopaedics. 2005; 16(4):236-9.
  12. McFarlane RM. Patterns of the diseased fascia in the fingers in Dupuytren's contracture. Displacement of the neurovascular bundle. Plastic and Reconstructive Surgery. 1974; 54(1):31-44.
  13. Luck JV. Dupuytren's contracture; a new concept of the pathogenesis correlated with surgical management. Journal of Bone and Joint Surgery. 1959; 41-A(4):635-64.
  14. Jemec B, Grobbelaar AO, Wilson GD, Smith PJ, Sanders R, McGrouther DA. Is Dupuytren's disease caused by an imbalance between proliferation and cell death?
    Journal of Hand Surgery. 1999;24(5):511-4.
  15. Hindocha S, Stanley JK, Watson S, Bayat A. Dupuytren's diathesis revisited: Evaluation of prognostic indicators for risk of disease recurrence. Journal of Hand Surgery. 2006; 31(10):1626-34.
  16. Lilly SI, Stern PJ. Simultaneous carpal tunnel release and Dupuytren's fasciectomy. Journal of Hand Surgery. 2010; 35(5):754-9.
  17. Denkler K. Options for severe proximal interphalangeal joint contractures in dupuytren contracture. Plastic and Reconstructive Surgery. 2012. 130(1):205e-206e.Denkler K. Options for severe proximal interphalangeal joint contractures in dupuytren contracture. Plastic and Reconstructive Surgery. 2012. 130(1):205e-206e.
  18. Foucher G, Medina J, Navarro R. Percutaneous needle aponeurotomy: complications and results. Journal of Hand Surgery. 2003; 28(5):427-31.
  19. Moermans JP. Long-term results after segmental aponeurectomy for Dupuytren's disease. Journal of Hand Surgery. 1996; 21(6):797-800.
  20. Anwar MU, Al Ghazal SK, Boome RS. Results of surgical treatment of Dupuytren's disease in women: a review of 109 consecutive patients. Journal of Hand Surgery. 2007; 32(9):1423-8.
  21. Mikkelsen OA. Dupuytren's disease--a study of the pattern of distribution and stage of contracture in the hand. Hand. 1976; 8(3):265-71.
  22. Zyluk A, Jagielski W. The effect of the severity of the Dupuytren's contracture on the function of the hand before and after surgery. Journal of Hand Surgery. 2007; 32(3):326-9.
  23. Donaldson OW, Pearson D, Reynolds R, Bhatia RK. The association between intraoperative correction of Dupuytren's disease and residual postoperative contracture. Journal of Hand Surgery. 2010; 35(3):220-3.

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Last updated: 2019-07-11 21:01