Dupuytren's Contracture

Dupuytren's Contracture

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Dupuytren's Contracture is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Palmar Fibromas
  • Palmar Fibromatosis, Familial
  • Plantar Fibromas
  • Plantar Fibromatosis, Familial

Disorder Subdivisions

  • None

General Discussion

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.

Symptoms

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.

Causes

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.

Affected Populations

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.

Standard Therapies

Diagnosis

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.



Treatment

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.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:491.



Berkow R., ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:397-98.



Royce PM, Steinmann B. Connective Tissue and Its Heritable Disorders. 2nd ed. Wiley-Liss, Inc., New York, NY. 2002:363.



REVIEW ARTICLES

McFarlane RM. On the origin and spread of Dupuytren's disease. J Hand Surg [Am]. 2002;27:385-90.



Frank PL. An update on Dupuytren's contracture. Hosp. Med. 2001;62:678-81.



JOURNAL ARTICLES

Wilbrand S, Ekbom A, Gerdin B. A cohort study linked increased mortality in patients treated surgically for Dupuytren's contracture. J Clin Epidemiol. 2005;58:68-74.



Augoff K, Kula J, Gosk J, et al. Epidermal growth factor in Dupuytren's disease. Plat Reconstr Surg. 2005;115:128-33.



Godtfredsen NS, Lucht H, Prescott E, et al. A prospective study linked both alcohol and tobacco to Dupuytren's disease. J Clin Epidemiol. 2004;57:858-63.



Draviaraj KP, Chakrabarti I. Functional outcome after surgery for Dupuytren's contracture: a prospective study. J Hand Surg [Am]. 2004;29:804-08.



Geoghegan JM, Forbes J, Clark DI, et al. Dupuytren's disease risk factors. J Hand Surg [Br]. 2004;29:423-26.



Qian A, Meals RA, Rajfer J, et al. Comparison of gene expression profiles between Peyronie's disease and Dupuytren's contracture. Urology. 2004;64:399-404.



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

Resources

March of Dimes Birth Defects Foundation

1275 Mamaroneck Avenue

White Plains, NY 10605

Tel: (914)997-4488

Fax: (914)997-4763

Tel: (888)663-4637

Email: Askus@marchofdimes.com

Internet: http://www.marchofdimes.com



NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases

Information Clearinghouse

One AMS Circle

Bethesda, MD 20892-3675

USA

Tel: (301)495-4484

Fax: (301)718-6366

Tel: (877)226-4267

TDD: (301)565-2966

Email: NIAMSinfo@mail.nih.gov

Internet: http://www.niams.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



Dupuytren Foundation

1732 S Congress Ave

Suite 348

Palm Springs, FL 33461

Tel: (949)287-3387

Fax: (561)828-0494

Email: info@DupuytrenFoundation.org

Internet: http://DupuytrenFoundation.org



For a Complete Report

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