Overview
Dupuytren's disease, also called Dupuytren’s contracture, is a progressive condition that causes the fingers to bend toward the palm at the first or second joint. The ability to straighten the fingers is lost, as is normal use of the hand. The medical term for this bending is “flexion contracture;” flexion means bending and contracture means thickening or shortening. The fingers most often affected are the small and ring fingers. The thumb is affected less often.
Severity varies widely. A single finger might be mildly contracted, or several fingers might be severely contracted. And the rate at which symptoms progress differs between patients. In many people, the disease is stable over a long period and requires only observation. In others, the condition advances rapidly and requires surgery.
The contractures are caused by lumps of tissue – “cords” of abnormal growth of the palmar fascia, which is the “gristle” like tissue that holds the skin of the palm in place. Dupuytren’s cords tighten over time, pulling the fingers toward the palm. As the condition progresses, the contracture can worsen; the patient can lose the ability to straighten the affected fingers. The lumps and cords can themselves be painful, too.
(Figure 1 shows the typical appearance of a hand affected by Dupuytren’s contracture.)
Similar conditions
A few conditions can be confused with Dupuytren's disease:
- “Trigger finger,” in which a finger’s flexor tendon (on the palm side) thickens and sticks in the tunnel of connective tissues in the wrist. This leads to finger(s) inability to fully open or close. It occurs more commonly in patients who also have Dupuytren's disease.
- Rupture of extensor tendon due to injury or arthritis. The extensor tendons (on the back of the hand, atop the fingers) enable you to straighten your fingers and thumb.
- Joint stiffness following injury and/or immobilization of the hand in a splint or cast.
- Joint stiffness due to arthritis.
- “Claw hand” deformity from injury to the ulnar nerve (which runs from the shoulder to the hand).
- Slipping of the extensor tendons between the knuckles in patients with arthritis.
None of these conditions results in the nodules and cords of the palm seen in Dupuytren's disease, making the clinical diagnosis of Dupuytren's disease relatively straightforward. However, Dupuytren's disease and one or more of these other conditions can be present in the same patient, which can cloud a diagnosis of Dupuytren’s disease.
Symptoms
Early on, the only sign might be a small lump of abnormal tissue in the palm, called a Dupuytren's nodule. The disease may remain stable, or nodes and/or cords may grow relatively quickly, involving one or all fingers and possibly the thumb.
Causes
Several different factors, including genetics, may be involved in the development of Dupuytren's disease.
Some patients are said to have a strong “Dupuytren's diathesis” (diathesis means predisposition or tendency). Thus, Dupuytren’s diathesis refers to patients with a strong family history of the condition.
They may also experience associated conditions such as Peyronie's disease (abnormal growth of the connective tissue in the penis), Ledderhosen disease (a condition similar to Dupuytren’s diseases affecting the soles of the feet) and knuckle pads (lumps of abnormal tissue over the knuckles). In these patients, Dupuytren's disease is often more aggressive.
Risk Factors
Dupuytren's disease is most common in Caucasian males older than 50. It occurs with less frequency among women and younger people. Prevalence of the diseases is higher among people with the following conditions:
- Diabetes
- HIV
- Hypothyroidism (an underactive thyroid gland)
- Seizure disorders
Dupuytren’s also is more frequent in people who smoke and drink alcohol, and who have blood relatives with the disease (especially of northern European ancestry). However, not every child of an affected individual will develop the disease, and not all patients with the disease will have an affected family member.
When several factors are known to be linked to a disease but none can be pinpointed as directly responsible, the cause is said to be “multifactorial.” This means that several things can increase a chance that a person will be affected, but a single cause is unknown.
Diagnosis
Usually patients notice an abnormal lump in their palm. A doctor diagnoses Dupuytren's disease based on the patient’s symptoms and a physical exam. No X-rays, other imaging studies or blood tests are required to make the diagnosis. The diagnosing physician often refers the patient to a hand surgeon, who can then work with the patient to determine whether surgery is needed.
Complications
Dupuytren's disease is not dangerous, but it can interfere with the patient's ability to use his or her hands, adversely affecting the ability to work and engage in many activities. In general, surgery is useful after the condition significantly affects the hand.
Recovery
Currently no medications can help to manage Dupuytren's disease.
Exercises have not been found to effectively treat the contractures associated with Dupuytren's disease. However, some surgeons might refer patients to a hand therapist for stretching of a severely involved finger prior to surgery.