Dupuytren Disease



Patient Story





A 53-year-old man presented with stiffness in his hands. He said his hands began to feel stiff several years ago, and now he finds that he cannot straighten many of his fingers (Figure 107-1). He delayed seeing a physician because he did not feel any pain in his hands. He recently began having difficulty holding his woodworking tools and wants to regain the function he has lost in his hands. The physician diagnosed him with Dupuytren contracture and discussed the disease with him along with his options for treatment.







Figure 107-1



Dupuytren contracture in a 53-year-old man showing flexion contractures at the proximal interphalangeal joints of the third digit and a palmar cord. (Courtesy of Richard P. Usatine, MD.)







Introduction





Dupuytren contracture is a flexion contracture of one or more of the fingers in the hand. Patients develop a progressive thickening of the palmar fascia, which causes the fingers to bend in toward the palm and limits extension. Diagnosis is clinical and the palpable nodules in the palm are considered diagnostic. Treatment has historically been surgical, but a new nonsurgical treatment with a collagenase has been approved.






Synonyms





Dupuytren disease, Dupuytren contractures, palmar fibromatosis, morbus Dupuytren, Ledderhose disease.






Epidemiology






  • Dupuytren contracture is an autosomal dominant disease with incomplete penetrance (Figure 107-2).
  • Higher prevalence among whites, particularly of Northern European descent. There is an increasing incidence related to aging.5
  • More common in men than women (approximately 6:1).1,3
  • Incidence in the United States is estimated to be approximately 3 per 10,000 adults with an estimated prevalence of 7%.
  • Higher incidence in people who use tobacco and alcohol or who have diabetes mellitus or epilepsy.2







Figure 107-2



Dupuytren contracture in a 60-year-old man showing a flexion contracture of the fifth digit and a palmar cord. All of his brothers have Dupuytren contractures. (Courtesy of Richard P. Usatine, MD.)







Etiology and Pathophysiology





Dupuytren contractures form in three stages:







  • Myofibroblasts in the palmar fascia proliferate to form nodules.
  • Myofibroblasts then align along the lines of tension, forming cords.
  • Tissue becomes acellular leaving thick cords of collagen that tighten resulting in flexion contractures at the metacarpal phalangeal joint, the proximal interphalangeal joint, and, occasionally, the distal interphalangeal joint.






Risk Factors






  • Tobacco use.
  • Alcohol consumption.
  • Epilepsy.
  • Diabetes mellitus.
  • Carpal tunnel syndrome.
  • History of manual labor.
  • History of hand injury.




Jun 5, 2016 | Posted by in GENERAL & FAMILY MEDICINE | Comments Off on Dupuytren Disease

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