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dupuytrens

Dupuytren's contracture

Introduction

  • a painless common benign fibroproliferative disorder that leads to the formation of the collagen knots and fibres in the palmar fascia which leads to progressive flexion deformity of the fingers (sparing the thumb and forefinger) in older people generally over the age of 50yrs
  • global prevalence is ~8% of adults but there is substantial geographic variation
  • described in 1831 when Baron Guillaume Dupuytren delivered a lecture on permanent retractions of the flexed fingers which was published under the title “Leçon sur la rétraction permanente des doigts.” The condition bears his name, despite the fact Felix Platter in 1680, Henry Cline, Jr., of St. Thomas’ Hospital in 1808, and Sir Astley Cooper in 1818 had already described a similar condition, with Cline specifically noting the involvement of the palmar fascia.
  • it is ~ 6x more common in men than women

Aetiology

  • appears to be multifactorial with genetic factors and environmental factors resulting in a T-cell mediated autoimmune response
  • there is increased prevalence if:
    • FH Dupuytren's contracture
      • appears to be more common in northern Europeans and in Africans
      • in a study in the French port of Toulon, 60% of the general population had brown eyes and 40% had blue eyes, but 80% of inhabitants with DD had blue eyes. The latter individuals were traced to the families of Breton and Norman sailors in the city’s history
      • a Canadian 1985 study of 812 patients, the family origin was Northern European in 68%, Southern European in 3%, black African and American Indian in 0.2%, Chinese in 2%, and Japanese in 16%; Japanese cases appear to have a different aetiology - in that 95% of cases occur in men and only 6% of cases occur in families with a history of DD—compared with 26% in other countries;
    • type 2 diabetes
    • alcohol abuse
    • smokers
    • occupations with heavy physical activity involving the hands
  • there have been a suggested increased prevalence in those with:
    • hypertension
    • COPD
    • TB
    • RhA
    • epilepsy
    • ischaemic heart disease
    • thyroid disease esp. Hashimoto’s thyroiditis
    • vitamin D defic?
    • HIV

Other associations

  • seems to be present in some 50% of patients with adhesive capsulitis (frozen shoulder or FS) and prevalence is 8x higher in these patients
    • FS is the third most common cause of musculoskeletal disability in the United States and affects patients between the ages of 40 and 60 years, with a prevalence rate of 2% to 5% 1)
    • bioinformatics analysis has identified 321 shared genes between frozen shoulder and Dupuytren's disease and revealed a protein-protein interaction (PPI) network, 15 hub genes and two immune-related candidate genes (POSTN and COL11A1) that may play crucial roles in both conditions 2)
    • there is growing evidence that autoimmune processes may be involved in both conditions
  • sometimes associated with other fibromatous conditions:
    • Peyronie's disease
    • plantar fibromatosis
    • fibromatosis of knuckle pads on dorsa of PIPjts - occur in up to 80% of pts with DD

Pathophysiology

  • it appears WNT signalling dysregulation may be a key driver:
    • downregulating WNT4 in normal skin fibroblasts leads to widespread 'DD like' changes in the transcriptome, suggesting WNT4 downregulation is a key driver of DD 3)

Mx

general non-surgical options

  • needle aponeurotomy
    • under LA, uses a needle to perforate and weaken the cords, followed by manipulation to rupture them
  • steroid injections
    • most useful in early stages
  • manual stretching under medical supervision
    • most useful in early stages

injectable collagenase

  • Xiaflex, a Clostridium histolyticum collagenase, has been used successfully with rapid results however it is non-PBS and was $AU1440 per vial but no longer available in Australia since 2021 apparently due to dramatic 7x price increases in the US leading to the distributor deciding to discontinue it in Australia as it became more expensive and less effective than open surgical Rx
    • each injection consists of a single dose of 0.58mg of Xiaflex into the cord affecting a primary joint.
    • incorrect injection into tendons, nerves or blood vessels may result in bleeding or damage and possible permanent injury to these structures.
    • lower clinical success rate (65%) compared to surgical fasciectomy (81%) and had higher rates of recurrence 4)

surgical options

  • surgical release is a mainstay of Rx for severe cases
dupuytrens.txt · Last modified: 2024/10/13 14:26 by gary1

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