DUPUYTREN'S DISEASE: what is it?

This a condition which mainly affects adult males, involving the palm of the hand and the fingers. Connective tissue (palmar fascia) right under the skin begins to thicken and shorten which causes development of contracted cords and nodules in the palm. It is the shortening and tightening of these cords that causes the fingers to draw down toward the palm. This is a genetic disease and is fairly common, most prevalent in men with northern European hereditary backgrounds. Onset of disease is usually in the fifth or sixth decade, with more aggressive forms starting at an  earlier age. A greater incidence of Dupuytren's contracture is found in individuals with epilepsy (where anticonvulsant medications suspected to be the stimulus), diabetes, alcoholism, HIV, and smoking. Trauma and exposure to chronic hand vibration may also exacerbate this condition.

SYMPTOMS

The disease usually begins with a palm nodule (can resemble a callus) that develops at the base of the ring or little finger. Gradually a prominent cord develops as the palmar fascia thickens. As the process continues the overlying skin puckers, dimples, and roughens. The thick cords contract slowly over time, drawing the fingers into the palm and may draw adjacent fingers together. The ring and little finger are most commonly affected and usually are affected first. Progression is often erratic and arbitrary with no obvious cause.

Associated conditions include the formation of pads on the top of the knuckles called "knuckle pads", fibrous plaques on the penis called "Peyronie's Disease", and a disease syndrome on the bottom of the feet, similar to what takes place in the palm, called "Ledderhose's disease".

TREATMENT

Needle Aponevrotomy or surgical intervention are the only methods available for correction of this disease. Aside from NA, non-surgical treatments have not been effective. Steroid injection into nodules may reduce pain, and traction devices may minimally undo contracture for the pre operative patient to achieve better surgical results.

Indication for treatment with either NA or surgery = CONTRACTIONS OF THE FINGERS. Combined flexion (drawing down) of the joints of the finger (MCP, PIP and DIP joint) exceeding 10-20 degrees should be released. There is no need to wait for The first joint of the finger (PIP) is more difficult to release than the large knuckle joint in the hand (MCP).

There is no cure for this disease. NA and surgery have the same rate of recurrence  - about 50% of patients experience recurrence of the disease within 2 or 3 years.

CURRENT METHODS OF TREATMENT

Needle Aponevrotomy (NA):  This is an outpatient procedure. A small hypodermic through a skin prick is used to divide and release the contracting bands.

Regional Fasciectomy: this is the most common surgical treatment of Dupuytren's contracture. This procedure completely excises the diseased fascia of the palm and digits. Requires general anesthesia or nerve block. Long rehab and wound care are needed.

Fasciotomy: Hand incision or multiple incisions are made above the hardened Dupuytren's cord and sharp dissection is performed to facilitate release. Diseased tissue is not removed.

Dermofasciectomy: Removal of diseased fascia as well as diseased skin overlying diseased fascia. This diseased skin is replaced with a skin graft taken from patients arm. Long rehab and wound care are needed, recurrence is somewhat less with this technique.

Non-invasive Pneumatic balloon extension procedure: a metal plate fitted to the palm. This plate secures a balloon and pneumatic assemblage where the patient can slowly increase the pressure to facilitate extension/traction. This device is primarily used to straighten severely contracted digits so that then will be more amenable to a surgical methods as described above.

OTHER CONSIDERATIONS

All of the following are controversial yet they possibly influence the course of Dupuytren's:

Citrus fruit, unsaturated fatty acids of native olive oil, and soybeans are all said to contain specific scavengers, which may give explanation to the lack in prevalence of Dupuytren's in the Mediterranean and Asian populations.

Vitamins A, C, D, E, and selenium may have a protective function.

Steroids, Allopurinol, and Colchicine may slow the fibro proliferation of the disease.

Interferon, Nifedipine and Verapmil are other drugs that were tried with limited success.

Collagenase: This enzyme is injected into diseased fascia causing it more or less to dissolve. Still in clinical trials but shows great promise.

Due to the rapid progress in biotechnology, someday we hope to be able to eradicate Dupuytren's disease through gene therapy.