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Dupuytren's contracture is a rare connective tissue disorder characterized by fixation of the joints (e.g., proximal interphalangeal joints and metacarpophalangeal joints) of certain fingers in a permanently flexed position (joint contractures). Due to abnormal thickening and shortening of the bands of fibrous tissue beneath the skin of the palm (palmar fascia), a hardened nodule may develop, eventually forming an abnormal band of hardened (fibrotic) tissue. As a result, the fingers of the affected area begin to be drawn in toward the palm over several months or years and cannot be pulled back (contracture). In addition, the skin of the affected area may pucker. In most cases, the ring and pinky (fourth and fifth) fingers are most affected. In addition, the disorder usually affects both hands (bilateral). Although the exact cause of Dupuytren's contracture is unknown, risk for the disorder appears to be increased by alcoholic liver disease (cirrhosis) and the presence or certain other diseases, including diabetes, thyroid problems, and epilepsy. In addition, it is thought that genetic predisposition may be a factor.
Dupuytren's contracture is characterized by a drawing up of the fingers toward the palms of the hand. The feet are rarely involved. Loss of function of the fingers and deformities may also occur, including nodular growths on the fingers. One or both hands may be affected. The right hand seems to be more frequently involved when involvement is only one sided (unilateral). The ring finger is involved most often, followed in order by the little, middle and index fingers. A nodule or plaque may develop as the first symptom on the finger. Symptoms may develop spontaneously and without any known associated condition. In other cases liver disease, alcoholism, pulmonary tuberculosis or diabetes mellitus may occur in conjunction with Dupuytren's contracture.
Dupuytren's contracture is a disease of the fibrous tissue. Its cause is unknown, but genetic predisposition and the presence of other diseases or medical conditions may be factors. It appears that one's risk is increased by certain lifestyle factors, such as alcoholism, and the presence of certain diseases, such as liver disease, diabetes, thyroid problems, and epilepsy.
Dupuytren's contracture is a rare disorder that affects more males than females by a ration of 4:1. The incidence of the disease increases after the age of 40. It also occurs with greater frequency in people who are chronically ill or have epilepsy, tuberculosis, or diabetes mellitus. People who have alcoholism also appear to be at increased risk for Dupuytren's contracture. It may also occur after a heart attack (myocardial infarction).
In the United States, Dupuytren's contracture occurs most often among males whose families have immigrated from Northern Europe. Incidence is lower among Afro-Americans and Asian-Americans.
Knuckle pads are nodules about the size of peas on the surface of the joints in the fingers (interphalangeal) consisting of new growths of fibrous tissue with thickening of the skin. They are probably of genetic origin.
Peyronie disease is a rare connective tissue disorder characterized by the development of fibrous plaques in the soft tissue of the penis of adult males. Affected individuals may experience pain, have cord-like lesions on the penis, and/or exhibit abnormal curvature of the penis when erect. In some cases, these conditions may make it impossible for many affected individuals to have normal sexual intercourse unless treated. Symptoms may be chronic, or may spontaneously resolve in some cases. The exact cause of Peyronie disease is not known. (For more information on this disorder, choose "Peyronie Disease" as your search term in the Rare Disease Database.)
The diagnosis of Dupuytren's contracture may be confirmed by a thorough clinical evaluation including a comprehensive patient history, observation of characteristic findings, and specialized tests. Histochemical tests and use of an electron microscope may confirm the diagnosis of Dupuytren's contracture.
Dupuytren's contracture is usually treated with corticosteroid injections into the affected tendon sheaths, analgesics for pain, and physical therapy. Surgery may be required according to the extent of the deformities. Recurrence is possible even after surgery.
Xiaflex injection is the first FDA-approved, nonsurgical option for the treatment of adult patients with Dupuytren's contracture with a palpable cord. When injected directly into a Dupuytren's cord, Xiaflex enzymatically disrupts collagen in the cord to help reduce the contracture and improve the range of motion.
Genetic counseling may be of benefit for patients and their families. Other treatment is symptomatic and supportive.
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FROM THE INTERNET
McKusick VA, ed. Online Mendelian Inheritance In Man (OMIM). The Johns Hopkins University. Dupuytren Contracture. Entry Number; 126900: Last Edit Date; 3/17/2004.
Freedman K. Dupuytren's contracture. MedlinePlus. Update date: August 3, 2004. www.nlm.nih.gov/medlineplus/ency/article/001233.htm
Report last updated: 2010/10/12 00:00:00 GMT+0