Dupuytren's contracture

Dupuytren's contracture is a condition in which one or more fingers become permanently bent
in a flexed position. It usually begins as small hard nodules just under the skin of the palm. It
then worsens over time until the fingers can no longer be straightened. While typically not
painful some aching or itching may be present. The ring finger followed by the little and middle
fingers are most commonly affected. It can interfere with preparing food, writing, and other
activities.
The cause is unknown. Risk factors include family history, alcoholism, smoking, thyroid
problems, liver disease, diabetes, previous hand trauma, and epilepsy. The underlying
mechanism involves the formation of abnormal connective tissue within the palmar fascia.
Diagnosis is usually based on symptoms.
Initial treatment is typically with steroid injections into the affected area and physical therapy.
Among those who worsen, clostridial collagenase injections or surgery may be
tried. While radiation therapy is used to treat this condition, the evidence for this use is poor.
The condition may recur despite treatment.

Sygns and symptoms

Dupuytren's most often occurs in males over the age of 50. It mostly affects white people and is
rare among Asians and Africans. In the United States about 5% of people are affected at some
point in time, while in Norway about 30% of men over 60 years old have the condition. In the
United Kingdom, about 20% of people over 65 have some form of the disease. It is named
after Guillaume Dupuytren, who first described the underlying mechanism in 1833.
Typically, Dupuytren's contracture first presents as a thickening or nodule in the palm, which
initially can be with or without pain. Later in the disease process, there is painless increasing
loss of range of motion of the affected fingers. 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. Generally, the cords or
contractures are painless, but, rarely, tenosynovitiscan occur and produce pain. The most
common finger to be affected is the ring finger; the thumb and index finger are much less often
affected.[8] The disease begins in the palm and moves towards the fingers, with the
metacarpophalangeal (MCP) joints affected before the proximal interphalangeal (PIP) joints.
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. People may report pain, aching and itching with the
contractions. Normally, the palmar fascia consists of collagen type I, but in Dupuytren sufferers,
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. Severe Dupuytren disease may also be associated with
frozen shoulder (adhesive capsulitis of shoulder), Peyronie's disease of the penis, increased risk
of several types of cancer, and risk of early death, but more research is needed to clarify these
relationships.

Risk factors
Dupuytren's contracture is a non-specific affliction but primarily affects:

Non-modifiable

 People of Scandinavian or Northern European ancestry, it has been called the "Viking
disease", though it is also widespread in some Mediterranean countries (e.g., Spainand
Bosnia) Dupuytren's is unusual among ethnic groups such as Chinese and Africans.
 Men rather than women; men are more likely to develop the condition)
 People over the age of 50; the likelihood of getting Dupuytren's disease increases with age
 People with a family history (60% to 70% of those afflicted have a genetic predisposition to
Dupuytren's contracture)

Modifiable

 Smokers, especially those who smoke 25 cigarettes or more a day

 Thinner people (i.e., those with a lower than average body mass index)
 Manual workers
 Alcoholics

Other conditions

 People with a higher-than-average fasting blood glucose level

 People with previous hand injury
 People with Ledderhose disease (plantar fibromatosis)
 People with epilepsy (possibly due to anti-convulsive medication)
 People with diabetes mellitus
 People with HIV
 In one study, those with stage 2 of the disease were found to have a slightly increased risk
of mortality, especially from cancer.

Diagnosis
According to the American Dupuytren's specialist Dr Charles Eaton, there may be three types of
Dupuytren's disease:

 Type 1: A very 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. According to Dr Eaton, this type may be made
more severe by other factors such as diabetes or heavy manual labour.
 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.
Treatment involves one or more different 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.
Needle aponeurotomy is most effective at "Stage I and Stage II" of 6–90 degrees of
deformation. However, it is also used at other stages.
Collagenase injection is most effective at "Stage I" and Stage II" of 6–90 degrees of
deformation. However, it is also used at other stages.
Hand surgery is effective at Stage I – Stage IV.

Surgery

On June 12, 1831, Dupuytren performed a surgical procedure on a person with contracture of
the 4th and 5th digits who had been previously told by other surgeons that the only remedy
was cutting the flexor tendons. He described the condition and the operation in The Lancet in
1834 after presenting it in 1833 and posthumously in 1836 in a French publication by Hôtel-
Dieu de Paris. The procedure he described was a minimally invasive needle procedure.
Because of high recurrence rates, new surgical techniques were introduced, such
as fasciectomy and then dermofasciectomy. Most of the diseased tissue is removed with these
procedures. For some individuals, the partial insertion of "K wires" into either the DIP or PIP
joint of the affected digit for a period of a least 21 days to fuse the joint is the only way to halt
the disease's progress. After removal of the wires, the joint is fixed into flexion, which is
considered preferable to fusion at extension.
In extreme cases, amputation of fingers may be needed for severe or recurrent cases or after
surgical complications.

Radiation therapy

Radiation therapy has been used mostly for early stage disease, but is unproven. Evidence to
support its use as of 2017, however, is poor; efforts to gather evidence are complicated due to
a poor understanding of the how the condition develops over time. It has only been looked at in
early disease.

Alternative medicine

Several alternate therapies such as vitamin E treatment, have been studied, although without
control groups. Most doctors do not value those treatments. None of these treatments stops or
cures the condition permanently.
Laser treatment (using red and infrared at low power) was informally discussed in 2013 at an
International Dupuytren Society forum, as of which time little or no formal evaluation of the
techniques had been completed.
Only anecdotal evidence supports other compounds such as vitamin E.

Prognosis
Dupuytren’s disease has a high recurrence rate, especially when a person has so called
Dupuytren’s diathesis. The term diathesis relates to certain features of Dupuytren's disease and
indicates an aggressive course of disease.
The presence of all new Dupuytren’s diathesis factors increases the risk of recurrent
Dupuytren’s disease by 71% compared with a baseline risk of 23% in people lacking the
factors. In another study the prognostic value of diathesis was evaluated. They concluded that
presence of diathesis can predict recurrence and extension. A scoring system was made to
evaluate the risk of recurrence and extension evaluating the following values: bilateral hand
involvement, little finger surgery, early onset of disease, plantar fibrosis, knuckle pads and
radial side involvement.
Minimally invasive therapies may precede higher recurrence rates. Recurrence lacks a
consensus definition. Furthermore, different standards and measurements follow from the
various definitions.
Postoperative care
Postoperative care involves hand therapy and splinting. Hand therapy is prescribed to optimize
post-surgical function and to prevent joint stiffness.
Besides hand therapy, many surgeons advise the use of static or dynamic splints after surgery
to maintain finger mobility. The splint is used to provide prolonged stretch to the healing
tissues and prevent flexion contractures. Although splinting is a widely used post-operative
intervention, evidence of its effectiveness is limited, leading to variation in splinting
approaches. Most surgeons use clinical experience to decide whether to splint. Cited
advantages include maintenance of finger extension and prevention of new flexion
contractures. Cited disadvantages include joint stiffness, prolonged pain, discomfort,
subsequently reduced function and edema.
A third approach emphasizes early self-exercise and stretching.