Dupuytren contracture is a myofibroblastic condition that causes one or more fingers to bend toward the palm of the hand. The affected fingers can't straighten completely due to knots of tissue that form under the skin. They eventually create a thick cord that can pull the fingers into a bent position. It most often affects the two fingers farthest from the thumb. This can complicate everyday activities such as placing your hands in your pockets, putting on gloves or shaking hands.
Dupuytren contracture gets worse slowly, over years. The condition begins with a firm lump in the palm of the hand. This lump might be a little tender, but usually isn't painful. Over time, the lump can extend into a hard cord under the skin and up into the finger. This cord tightens and pulls the finger toward the palm, sometimes severely.
Dupuytren disease is a genetic disorder that often is inherited in an autosomal dominant fashion, but is most frequently seen with a multifactorial etiology. It is associated with diabetes, seizure disorders, smoking, alcoholism, HIV, and vascular disease. Occupation and activities have not been shown to be risk factors.
The pathophysiology of Dupuytren disease involves abnormal myofibroblastic growth in the hand, predominantly composed of type III collagen. Numerous cytokines are involved including interleukin-1, transforming growth factor beta-1, transforming growth factor beta-2, epidermal growth factor, platelet-derived growth factor, and connective tissue growth factor.
Dupuytren contracture progresses through three phases: (1) proliferative, (2) involution, and (3) residual. The proliferative phase has a characteristically high concentration of immature myofibroblasts and fibroblasts arranged in a whorled pattern. In the involution phase, fibroblasts become aligned in the longitudinal axis of the hand following lines of tension. In the residual phase, relatively acellular collagen-rich chords remain causing contracture deformity.
Diabetes, as an example of the pathogenesis of Dupuytren disease, has long been considered a risk factor for Dupuytren disease. One of the proposed biochemical processes is the formation of advanced glycated end products (AGEs). AGEs have been associated with other fibroproliferative disorders, such as diabetic cardiomyopathy and idiopathic pulmonary fibrosis, and carpal tunnel syndrome. Data from biopsies from the palmar fascia in patients with diabetes showed higher levels of AGEs compared to a control group, which possibly may cause increasing levels of collagen deposition and increased collagen stiffness.3 Furthermore, studies have also shown that diabetes can cause increased formation of myofibroblasts, one of the proposed main cell types responsible for contraction the finger. The study has confirmed that participating patients with diabetes at baseline had marked increased risk for development of Dupuytren disease during follow-up; even when adjusting for other known risk factors, e.g. alcohol consumption and BMI.
