Dupuytren contracture

Dupuytren contracture is a common fibroproliferative disorder that affects the palmar fascia, mainly of the 4th and 5th fingers, and more commonly affects men than women. The exact cause is unknown. Trauma (e.g., from manual labor, pneumatic tools) or ischemic injury (e.g., from cigarette smoking, diabetes) are thought to stimulate fibroblast proliferation and collagen deposition in the palmar fascia of genetically susceptible individuals. Early signs include palpable palmar nodules or skin thickening, puckering, or pitting adjacent to the distal palmar crease. As the disease progresses, thick palmar cords develop, limiting extension of the affected digits, eventually leading to flexion contractures. Diagnosis is usually clinical. Management depends on disease severity; options for patients with mild disease include observation, as disease progression is often slow and may even regress, and intralesional steroid injections. For patients with functional impairment or significant flexion contractures, intervention such as intralesional collagenase injections, needle aponeurotomy, or surgery is recommended. Recurrence is common.

• Prevalence: 4–6% ^[1][2]
• Peak incidence: 40–60 years ^[3]
• Sex: ♂ > ♀ ^[4]

Epidemiological data refers to the US, unless otherwise specified.

The exact etiology is unknown, but several factors appear to play a role in the development of the disease.

• Genetic predisposition: ∼ 70% of patients have a positive family history. ^[5]
• Autoimmunity: There is evidence of T-cell accumulation in the fibrotic tissue from the contracture sites, which suggests a possible autoimmune origin of the disease. ^[6][7]
• Risk factors: these factors may cause ischemic injury of the palmar fascia with subsequent development of Dupuytren contracture in genetically predisposed individuals ^[4][8][9]
  • Cigarette smoking
  • Recurrent trauma; (e.g., use of pneumatic tools used in construction, manual labor)
  • Diabetes
  • Heavy alcohol use ^[8]

• Dupuytren contracture (palmar fibromatosis) is a fibroproliferative disorder of the palmar fascia ^[10]
• Injury (trauma/ischemia) to the palmar fascia triggers myofibroblasts → fibroblast proliferation and collagen (collagen type III) deposition ; → thickening of the palmar fascia → formation of nodules in the palmar fascia
• The nodules are adherent to the overlying dermis → characteristic puckering of palmar skin ^[11]
• Nodules progress to form cords in the palmar fascia → flexion contractures ; of the palmar fascia

Dupuytren contracture typically manifests as painless palmar contractures, most commonly affecting the 4th and 5th fingers. Bilateral involvement is common. ^[9][11]

• Early disease ^[9][12]
  • Palpable palmar nodule or skin thickening, puckering, or pitting adjacent to the distal palmar crease
  • Function is not usually impaired.
• Advanced disease ^[9][12]
  • Palpable palmar cord
  • Limited finger extension
  • Flexion contractures of the metacarpophalangeal joint (MCP) and the proximal interphalangeal joint (PIP) of the affected digits
  • Restricted activities of daily living (e.g., difficulty dressing, combing hair, washing, or putting hands in pockets)
• Signs of concurrent fibromatoses may be present, e.g.: ^[12]
  • Knuckle pads (Garrod nodules)
    • Fibrofatty pads over the dorsal aspect of the PIPs
    • May be tender
    • Associated with aggressive disease ^[12]
  • Plantar fibromatosis (Ledderhose disease)
    • Plantar equivalent of Dupuytren contracture
    • Characterized by fibrosis of the plantar fascia
  • Peyronie disease

• Diagnosis is usually clinical. ^[13]
• Table top test ^[11][14]
  • Ask the patient to place the palm flat against a table top.
  • Inability to do so indicates flexion contractures of the affected digits (advanced disease).
• Ultrasound
  • Not routinely performed; may be used to guide intralesional therapy ^[10]
  • Findings include nodules in the palmar fascia superficial to the flexor tendons. ^[15]
• Consider further diagnostics as needed to identify underlying risk factors (e.g., screening for diabetes). ^[11]

The differential diagnoses listed here are not exhaustive.

General principles ^[12][18]

• Treatment depends on disease stage.
  • Conservative measures may be considered in early disease before there is functional impairment.
  • Refer patients with advanced disease or functional impairment to a specialist (e.g., hand surgeon) for intervention.
• Manage modifiable risk factors, e.g.:
  • Counsel on smoking cessation.
  • Counsel on alcohol use disorder.
  • Manage diabetes.

Early disease ^[12][18]

• Observation
• Physiotherapy and splinting
• Intralesional steroid injections
  • Can flatten and soften nodules ^[18]
  • Does not limit disease progression ^[12]
• Radiotherapy: emerging therapy to prevent disease progression ^[12][18][19]

Advanced disease

• Refer to a specialist for intervention if any of the following are present: ^[9][12]
  • Functional impairment
  • Positive table top test
  • Progressive contractures
  • MCP flexion deformity ≥ 30°
  • PIP flexion deformity ≥ 15°
• Choice of intervention depends on contracture severity and patient tolerance for symptom recurrence. ^[11][12]
  • Intralesional collagenase Clostridium histolyticum injections
  • Needle aponeurotomy
  • Surgery; (limited or radical fasciectomy): gold standard for severe contractures ^[12]

• Variable prognosis ^[9][12]
  • Can remain indolent or progress
  • Regression occurs in approximately 10% of patients. ^[9]
  • Recurrence rates are high, even after surgery (∼ 60%). ^[10]
• Patients with the following risk factors (known as Dupuytren diathesis) have more aggressive disease and higher rates of recurrence. ^[12]
  • Age of onset < 50 years
  • Male sex
  • Bilateral disease
  • Northern European descent
  • Knuckle pads