Dupuytren Contracture
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Basics
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
-- To view the remaining sections of this topic, please log in or purchase a subscription --
Basics
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
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