Dupuytren's contracture (also called Dupuytren's disease, Morbus Dupuytren, Palmar fibromatosis and colloquially Viking disease, Viking hand or Celtic hand) is a condition in which one or more fingers become permanently bent in a flexed position. It is named after Guillaume Dupuytren, who first described the underlying mechanism of action, followed by the first successful operation in 1831 and publication of the results in The Lancet in 1834. It usually begins as small, hard nodules just under the skin of the palm, then worsens over time until the fingers can no longer be fully straightened. While typically not painful, some aching or itching, or pain, may be present. The ring finger followed by the little and middle fingers are most commonly affected. It can affect one or both hands. The condition can interfere with activities such as preparing food, writing, putting the hand in a tight pocket, putting on gloves, or shaking hands.
The causes of Dupuytren's contracture are not completely understood. The disease is driven by over-activation of fibroblasts and myofibroblasts in response to signaling molecules including IL-1 beta, TGF-beta, EGF, and CTGF, leading to inappropriately increased formation of fibrous connective tissue. Risk factors include family history, smoking, thyroid problems, liver disease, diabetes, previous hand trauma, and epilepsy. The development of Dupuytren disease has also been associated with environmental factors, including smoking, alcohol consumption, aging, trauma, and repetitive use of the hand in certain physical occupations. Genetic studies have implicated mutations in the Wnt signaling pathway a possible hereditary genetic contributor to the disease, and various somatic chromosomal abnormalities as a non-hereditary or partially hereditary contributing factor.
In 2020, the World Health Organization reclassified Dupuytren's (termed palmar-type fibromatosis) as a specific type of tumor in the category of intermediate (locally aggressive) fibroblastic and myofibroblastic tumors.
Initial treatment is typically with cortisone injected into the affected area, occupational therapy, and physical therapy. Among those who worsen, clostridial collagenase injections or surgery may be tried. Radiation therapy may be used to treat this condition. The Royal College of Radiologists (RCR) Faculty of Clinical Oncology concluded that radiotherapy is effective in early stage disease which has progressed within the last 6 to 12 months. The condition may recur at some time after treatment; it can then be treated again. It is easier to treat when the amount of finger bending is more mild.
It was once believed that Dupuytren's most often occurred in white males over the age of 50 and was thought to be rare among Asians and Africans. It sometimes was called "Viking disease," since it was often recorded among those of Nordic descent. In Norway, about 30% of men over 60 years old have the condition, while in the United States about 5% of people are affected at some point in time. In the United Kingdom, about 20% of people over 65 have some form of the disease.
More recent and wider studies show the highest prevalence in Africa (17 percent) and Asia (15 percent).
Signs and symptoms
Typically, Dupuytren's contracture first presents as a thickening or nodule in the palm, which initially can be with or without pain. Page last reviewed: 29/05/2015 Later in the disease process, which can be years later, there is increasing loss of range of motion of the affected finger(s). The earliest sign of a contracture is a triangular "puckering" of the skin of the palm as it passes over the flexor tendon just before the flexor crease of the finger, at the metacarpophalangeal (MCP) joint.
Dupuytren disease is generally considered painless, but can be painful if nerve tissue is involved, although this is not usually discussed in the literature. The most common finger to be affected is the ring finger; the thumb and index finger are much less often affected. The disease begins in the palm and moves towards the fingers, with the metacarpophalangeal (MCP) joints affected before the proximal interphalangeal (PIP) joints. The MCP joints at the base of the finger responds much better to treatment and are usually able to fully extend after treatment. Due to anatomic differences in the ligaments and extensor tendons at the PIP joints, they may have some residual flexion. Proper patient education is necessary to set realistic treatment expectation. In Dupuytren's contracture, the palmar fascia within the hand becomes abnormally thick, which can cause the fingers to curl and can impair finger function. The main function of the palmar fascia is to increase grip strength; thus, over time, Dupuytren's contracture decreases a person's ability to hold objects and use the hand in many different activities. Dupuytren's contracture can also be experienced as embarrassing in social situations and can affect quality of life. People may report pain, aching, and itching with the contractions. Normally, the palmar fascia consists of collagen type I, but in Dupuytren patients, the collagen changes to collagen type III, which is significantly thicker than collagen type I.
Related conditions People with severe involvement often show lumps on the back of their finger joints (called "Garrod's pads", "knuckle pads", or "dorsal Dupuytren nodules"), and lumps in the arch of the feet (plantar fibromatosis or Ledderhose disease). In severe cases, the area where the palm meets the wrist may develop lumps. It is thought the condition Peyronie's disease is related to Dupuytren's contracture.
In one study those with stage 2 of the disease were found to have a slightly increased risk of mortality, especially from cancer.
Risk factors Many risk factors have been suggested or identified:
Non-modifiable Scandinavian or Northern European ancestry; Dupuytren's has been called the "Viking disease", though it is also widespread in some Mediterranean countries, e.g., Spain and Bosnia. Dupuytren's is uncommon among groups including Chinese and Africans. In June 2023 a study found that gene variants that were inherited from Neanderthals dramatically increased the odds of developing the condition Male sex; men are 80% more likely to develop the condition Age of 50 or over (5% to 15% of men in that group in the US); the likelihood of getting Dupuytren's disease increases with age A family history (60% to 70% of those affected have a genetic predisposition to Dupuytren's contracture)
Modifiable Smoking, especially 25-plus cigarettes per day Lower-than-average body mass index (thinness).
Manual work: a 2023 paper by researchers at the University of Groningen Medical Centre and Oxford University, "Dupuytren's disease is a work-related disorder: results of a population-based cohort study", found that people whose jobs involved significant manual work were 1.29 times more likely to develop Dupuytren's disease than others, with a linear dose–response relationship with cumulative manual labour over 30 years.
Other conditions Previous hand injury Ledderhose disease (plantar fibromatosis) Epilepsy (possibly due to anti-convulsive medication)"Etiology of Dupuytren's Disease" Living Textbook of Hand Surgery. Higher-than-average fasting blood glucose level Diabetes mellitus HIV Macrophallism Previous myocardial infarction
Pathophysiology Dupuytren’s contracture is a fibroproliferative disorder of the palmar fascia in which abnormal activation of fibroblasts and myofibroblasts, driven by mediators such as transforming growth factor-beta, platelet-derived growth factor, epidermal growth factor, interleukin-1 beta, and connective tissue growth factor, leads to excess deposition of type III collagen and progressive remodeling of fascial tissue. Studies have suggested that intracellular signaling, as opposed to paracrine or endocrine signaling, may be the strongest driver of abnormal fibroblast activity in most cases of Dupuytren's contracture.
The disease typically evolves through a proliferative stage marked by cellular nodules rich in immature fibroblasts and myofibroblasts, an involution stage in which these cells align along longitudinal stress lines in the hand, and a residual stage in which dense, relatively hypocellular collagenous cords persist and mechanically flex the digits. As normal fascial structures are converted into pathologic cords, characteristic deformities emerge: central cords commonly produce skin puckering and metacarpophalangeal contracture, natatory cords (developed from the natatory ligament) narrow the web spaces, and spiral cords can cause proximal interphalangeal contracture while displacing the digital neurovascular bundle. Although the condition may remain stable or even regress in many patients, greater severity and recurrence are associated with strong hereditary influence and related fibromatoses, and genetic studies increasingly implicate dysregulated Wnt signaling as an important contributor to disease susceptibility and progression.
Diagnosis Types
There may be three types of Dupuytren's disease: Type 1: An aggressive form of the disease found in only 3% of people with Dupuytren's, which can affect men under 50 with a family history of Dupuytren's. It is often associated with other symptoms such as knuckle pads and Ledderhose disease. This type is sometimes known as Dupuytren's diathesis. Type 2: The more normal type of Dupuytren's disease, usually found in the palm only, and which generally begins above the age of 50. This type may be made more severe by other factors such as diabetes or heavy manual labor. Type 3: A mild form of Dupuytren's which is common among diabetics or which may also be caused by certain medications, such as the anti-convulsants taken by people with epilepsy. This type does not lead to full contracture of the fingers, and is probably not inherited.
Treatment Treatment is indicated when the so-called table-top test is positive. With this test, the person places their hand on a table. If the hand lies completely flat on the table, the test is considered negative. If the hand cannot be placed completely flat on the table, leaving a space between the table and a part of the hand as big as the diameter of a ballpoint pen, the test is considered positive and surgery or other treatment may be indicated. Additionally, finger joints may become fixed and rigid. There are several types of treatment, with some hands needing repeated treatment.
The main categories listed by the International Dupuytren Society in order of stage of disease are radiation therapy, needle aponeurotomy (NA), collagenase injection, and hand surgery. the evidence on the efficacy of radiation therapy was considered inadequate in quantity and quality, and difficult to interpret because of uncertainty about the natural history of Dupuytren's disease.
Needle aponeurotomy is most effective for Stages I and II, covering 6–90 degrees of deformation of the finger. However, it is also used at other stages. Collagenase injection is likewise most effective for Stages I and II. However, it is also used at other stages.
Hand surgery is effective at stage I to stage IV.
Use of a splint to keep treated fingers straight following various forms of treatment, typically at all times for some days, then at nighttime for some weeks, is usual. However, a 2015 Cochrane review concluded: "low-quality evidence suggests that postoperative splinting may not improve outcomes and may impair outcomes by reducing active flexion. Further trials on this topic are urgently required".
Surgery