Dupuytren's Contracture Disease

BASICS

DESCRIPTION

Contracture of the palmar fascia due to fibrous proliferation resulting in flexion deformities and loss of function. Similar change may rarely occur in plantar fascia. It usually appears simultaneously.

  • System(s) affected: Musculoskeletal
  • Genetics:
    • Autosomal dominant with variable penetrance
    • 10% of patients have a positive family history
  • Incidence/Prevalence in USA:
    • Unknown
    • Norway - 9% males and 3% females
  • Predominant age: 50 for males; 60 for females
  • Predominant sex: Male > Female (ranges from 2:1 to 10:1)
SIGNS AND SYMPTOMS
  • Typical
    • Caucasian male 50-60
    • Bilateral with one hand more involved
    • Family history
    • Unilateral or bilateral (50%)
    • Right hand more frequent
    • Ring finger more frequent
    • Ulnar digits more affected than radial
    • Mild pain early
    • Later painless plaques or nodules in palmar fascia
    • Extends into a cord-like band in the palmar fascia
    • Skin adheres to fascia and becomes puckered
    • Nodules can be palpated under the skin
    • Digital fascia becomes involved as disease progresses
    • Web space contractures
    • Dupuytren's diathesis can involve plantar (Ledderhose's - 10%) and penile (Peyronie's - 2%) fascia
    • Knuckle pads
  • Atypical
    • No age, gender differences
    • No family history
    • May have systemic disease (see Risk Factors)
    • May have a history of trauma
    • More common unilateral
    • No ectopic manifestations (Ledderhose's or Peyronie's)
    • Nonprogressive
CAUSES
  • Unknown
  • Ischemia to the fascia with oxygen free radical formation
  • Possibly related to release of angiogenic basic fibroblast growth factor
  • Related to microhemorrhage and release of growth factors
RISK FACTORS
  • Smoking (mean 16 pack-years, odds ratio 2.8)
  • Alcohol intake
  • Increasing age
  • Male/Caucasian
  • Diabetes mellitus (one-third affected, increases with time, usually mild; middle and ring finger involved)
  • Epilepsy
  • Chronic illness (e.g., pulmonary tuberculosis, liver disease)
  • Hypercholesterolemia
  • Liver disease
  • HIV infection

DIAGNOSIS

LABORATORY

N/A

Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS
  • Myofibroblasts
  • First stage (proliferative) - increased myofibroblasts
  • Second stage (residual) - dense fibroblast network
  • Third stage (involutional) - myofibroblasts disappear
SPECIAL TESTS
N/A
IMAGING
MR can assess cellularity of lesions which correlate with higher recurrence after surgery
DIAGNOSTIC PROCEDURES
N/A

TREATMENT

APPROPRIATE HEALTH CARE
  • Outpatient monitoring and physical therapy
  • Inpatient if surgery indicated
GENERAL MEASURES
  • Steroid injection for acute tender nodule
  • Physiotherapy is ineffective alone
  • Isolated involvement of palmar fascia can be followed
  • Metacarpophalangeal (MP) joint involvement can be followed if flexion contracture is < 30 degrees
SURGICAL MEASURES
  • Surgery - selective fascial ray release
    • Indications: Any involvement of the proximal interphalangeal (PIP) joints. Hueston's table-top test, if positive, consider surgery (when the palm is placed on a flat surface, the digits can not be simultaneously placed fully on the same surface as the palm because of flexion contractures).
    • May require skin grafts for wound closure with severe cutaneous shrinkage
    • 80% have full range of movement if operated on early
    • Continuous elongation technique is useful to prepare a severely contracted PIP joint for surgery. The digit can frequently be completely extended, however, will relapse if surgery not performed.
    • Amputation of little finger, if severe and deforming
ACTIVITY
  • No restrictions
  • Physical therapy after surgery - started 3-5 days after surgery (passive and active exercises, posterior dynamic extension splints)
DIET

No special diet

PATIENT EDUCATION
  • Avoid risk factors especially with a strong family history
  • Regular follow-up by physician every 6 months-1 year

FOLLOW UP

PREVENTION/AVOIDANCE

None known. Avoid risk factors when possible.

POSSIBLE COMPLICATIONS
  • Postsurgery development of reflex sympathetic dystrophy
  • Postoperative recurrence or extension 46-80%
  • Postoperative hand edema and skin necrosis
  • Digital infarction
EXPECTED COURSE AND PROGNOSIS
  • Typical
    • Unpredictable, but usually slowly progressive
    • Patients likely to have aggressive disease (one or more) < 40 at onset, knuckle pads, positive family history, bilateral disease involving radial side of hand
    • Reports of clinical regression with continuous passive skeletal traction in extension and under a skin graft
    • Recurrence rate after surgery is 10-34%
    • Prognosis better for MP joint vs PIP joint after surgery
  • Atypical
    • Nonprogressive
    • Surgery rarely needed
    • Recurrence unlikely if surgery performed

MISCELLANEOUS

ASSOCIATED CONDITIONS
  • Alcoholism
  • Epilepsy
  • Diabetes mellitus
  • Chronic lung disease
  • Occupational hand trauma (vibration white finger)
  • Shoulder-hand syndrome
  • Status post myocardial infarction
  • Hypercholesterolemia
AGE-RELATED FACTORS

Pediatric: N/A
Geriatric: Primarily in this age group
Others: N/A

PREGNANCY

N/A

OTHER NOTES

N/A

ABBREVIATIONS

MP = Metacarpophalangeal
PIP = proximal interphalangeal

Clinical Investigations

ROLE OF HOMOEOPATHY

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