Dupuytren Contracture

BASICS

DESCRIPTION

  • Palmar fibromatosis is caused by progressive fibrosis of the palmar fascia, leading to flexion deformities and functional loss.
  • Distinct from “trigger finger,” which results from thickening of the distal flexor tendon.
  • Similar change rarely occurs in plantar fascia, usually appearing simultaneously.
  • System(s) affected: musculoskeletal
  • Dupuytren diathesis is an aggressive heritable form associated with age of onset <40 years, bilateral presentation to include radial digits, plantar fibromatosis (Ledderhose disease), and penile fibromatosis (Peyronie disease) (1).
  • Synonyms: morbus Dupuytren; Dupuytren disease; “Celtic hand”; Viking disease; palmar fascial fibromatosis, contracture of palmar fascia

EPIDEMIOLOGY

Prevalence

  • Increases with age; mean prevalence in western countries: 12%, 21%, and 29% at ages 55, 65, and 75 years, respectively; Norway: 30% of males aged >60 years; Spain: 19% of males aged >60 years
  • More common in Caucasian men of Scandinavian or Northern European ancestry
  • Mean age of onset is 60 years with typical age-range of onset between 40 and 80 years.

ETIOLOGY AND PATHOPHYSIOLOGY

  • Definitive etiology unknown; possibly oxidative stress, altered wound repair, and/or abnormal immune response
  • Progresses through three stages (Luck classification) (1):
    • Proliferative phase: proliferation of myofibroblasts with thickening of the palmar fascia and nodule development on palmar surface
    • Involutional stage: Myofibroblasts spread along palmar fascia to fingers with cord development via production of more type 3 collagen.
    • Residual phase: Fibroblasts are predominant with dense collagen leading to cord tightening and contracture flexion formation.

Genetics

  • Autosomal dominant inheritance with incomplete penetrance:
    • Siblings with 3-fold risk
  • 68% of male relatives of affected patients develop disease at some time.
  • Possible association with HLA alleles

RISK FACTORS

  • Smoking (mean 16 pack-years, odds ratio: 2.8)
  • Increasing age
  • Male/Caucasian; male > female (range 3.5:1 to 9:1)
  • Vibration exposure and manual work—risk doubles if regular (weekly) exposure
  • Diabetes mellitus (DM) (increases with duration of DM, usually mild; middle and ring fingers are involved)
  • Excessive alcohol consumption
  • Cirrhosis
  • Northern European ethnicity
  • Family history
  • Hand trauma
  • Low body weight and BMI

COMMONLY ASSOCIATED CONDITIONS

  • Alcoholism
  • Epilepsy (inconstant data)
  • DM, particularly type 1 DM
  • Chronic lung disease
  • Occupational hand trauma (vibration)
  • Hypercholesterolemia
  • Carpal tunnel syndrome
  • Peyronie disease
  • HIV
  • Cancer
  • Adhesive capsulitis of shoulder

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