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Dupuytren's diathesis
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Dupuytren's contracture is a visible and palpable palmar skin changes (pits, nodules) caused by thickening of the palmar fascia with cord like structures extending from the palm into the digits resulting in permanent flexion and/or adduction contracture of the metacarpo-phalangeal joint. It may also progress to involve the digital inter-phalangeal joints as well (1).

  • most commonly affected fingers (in decreasing order) are the fourth, fifth, third, and second (2).
  • it is often bilateral and may affect the soles of the feet
  • there is a nodular thickening of the connective tissue over the 4th and the 5th fingers

The prevalence of the disease may differ (from 2% to 42%) according to the selected population and increases with age and where there is a positive family history.

The disease is thought to affect more than 2 million people in UK (1).

  • Dupuytren's disease typically affects elderly men of northern European descent
    • a strong familial component is recognised, and the pattern of inheritance has been suggested to be autosomal dominant with variable penetrance
  • men typically present earlier (mean age 55 years) than women (10 years later) and have more severe disease (1).

The disease is uncommon in people from southern European or South American countries and is rarely seen in African countries and in China (2).

There is often associated with involvement of other areas of the body, so called ectopic disease

  • Garrod's knuckle pads (44-54%), plantar fibromatosis (Ledderhose disease, 6-31%), and penile fibromatosis (Peyronie's disease, 2-8%)
    • more aggressive form of the disease is known as Dupuytren's diathesis (1) - "...A diathesis is recognised in Dupuytren's disease and describes disease affecting patients younger than 50 years, white men with a strong family history, bilateral involvement, severe disease, and ectopic manifestations. Recognition of this clinical type is essential, as it carries a more serious prognosis and warrants aggressive follow-up and treatment...(1,3)"

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