Abstract
Dupuytren disease (DD) is a fibroproliferative disorder of unknown etiology that often results in shortening and thickening of the palmar fascia, leading to permanent and irreversible flexion contracture of the digits. This Review provides a detailed update of the scientific understanding of DD and its clinical management, with perspectives on emerging research and therapy. Established risk factors include genetic predisposition and ethnicity, as well as sex and age. Several environmental risk factors (some considered controversial) include smoking, alcohol intake, trauma, diabetes, epilepsy and use of anticonvulsant drugs, and exposure to vibration. DD has been variously attributed to the presence of oxygen free radicals, trauma to the palmar fascia, or aberrant immune responses with altered antigen presentation, or to interactions between these proposed mechanisms. The presence of immune cells and related phenomena in DD-affected tissue suggests that DD is possibly immune-related. Mechanically, digital contracture is caused by myofibroblasts in the DD palmar fascia; however, the exact origin of this cell type remains unknown. The mainstay of treatment is surgical release or excision of the affected palmodigital tissue, but symptoms often recur. Nonsurgical correction of DD contractures can be achieved by Clostridium histolyticum collagenase injection, although the long-term safety and recurrence rate of this procedure requires further assessment.
Key Points
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Genetic susceptibility, age, and ethnicity are the main risk factors for Dupuytren disease (DD); several environmental risk factors have also been implicated, although the evidence for some is controversial
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Epidemiological, familial, twin, and case–control studies support a genetic association for DD, and have identified susceptibility loci or genes, including those encoding transcription factor Zf9, mitochondrial 16s ribosomal RNA and HLA-DR alleles
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Several molecular aberrations have been observed in DD-affected tissue, relating to cytokines and growth factors, extracellular matrix proteins and associated molecules, and matrix metalloproteinases and associated proteins
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The pathophysiological mechanisms of DD are incompletely understood, but may be related to oxidative stress, altered wound repair and/or an aberrant immune response
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The mainstay of treatment for DD is surgery to relieve digital contractures, but this invasive intervention is associated with a high rate of disease recurrence
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Injection of Clostridium histolyticum collagenase effectively resolves DD contractures; however, the long-term recurrence rate and safety of this nonsurgical approach remain unclear
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B. Shih and A. Bayat contributed equally to researching data for the article, discussion of content, writing and reviewing/editing of the manuscript before submission.
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Shih, B., Bayat, A. Scientific understanding and clinical management of Dupuytren disease. Nat Rev Rheumatol 6, 715–726 (2010). https://doi.org/10.1038/nrrheum.2010.180
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DOI: https://doi.org/10.1038/nrrheum.2010.180
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