Description

• Palmar fibromatosis; caused by progressive fibrous proliferation and tightening of the fascia of the palms, resulting in flexion deformities and loss of function
• Not the same as “trigger finger,” which is caused by 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, bilateral presentation to include radial digits, plantar fibromatosis (Ledderhose), and penile fibromatosis (Peyronie) (1).
• Synonyms: morbus Dupuytren; Dupuytren disease; “Celtic hand”; Viking’s 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 >60 years; Spain: 19% of males >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
• Occurs in three stages (Luck classification) (1):
  • Proliferative phase: proliferation of myofibroblasts with 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 formation

Genetics

• Autosomal dominant 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 (increases with duration of DM, usually mild; middle and ring finger involved)
• Excessive alcohol consumption
• Northern European ethnicity
• Family history
• Hand trauma
• Low body weight and BMI

General Prevention

Avoid risk factors, especially if a strong family history.

Commonly Associated Conditions

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