Dupuytren contracture

Dupuytren contracture is a common fibroproliferative disorder affecting the palmar fascia mainly of the 4^th and 5^th fingers, particularly in males. The etiology is uncertain. Trauma (manual labor, pneumatic tools) or ischemic injury (cigarette smoking, diabetes) are thought to stimulate fibroblast proliferation and collagen deposition in the palmar fascia of genetically susceptible individuals. Skin puckering proximal to the flexor crease of the affected finger is the earliest sign. As the disease progresses, nodules and cords develop in the palmar fascia, causing flexion contractures to develop at the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints. Patients with Dupuytren contracture may also have fibroproliferative disorders at other sites (e.g., plantar fibromatosis and Peyronie disease). Diagnosis is usually clinical but ultrasound can demonstrate the nodules and cords of the palmar fascia. Treatment depends on the severity of the disease. Observation, passive finger extension exercises, and/or splints are useful in early disease. Patients with moderately severe contractures can be treated with intralesional injections of corticosteroid or collagenase. Severe contractures are best treated with surgery (fasciectomy, fasciotomy). Prognosis is variable, with the disease remaining indolent for many years or progressing rapidly. Regression is seen in approx. 10% of patients, and recurrence rates are high (approx. 60%) regardless of treatment.

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

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. ^[4]

• Genetic predisposition: ∼ 70% of patients have a positive family history. ^[5]
• Risk factors: these factors may cause ischemic injury of the palmar fascia with subsequent development of Dupuytren contracture in genetically predisposed individuals ^[4]
  • Cigarette smoking
  • Recurrent trauma; (e.g., use of pneumatic tools used in construction, manual labor)
  • Diabetes
  • Alcohol abuse ^[6]
  • Liver cirrhosis ^[6]
• 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. ^[7][8]

• Dupuytren contracture (palmar fibromatosis) is a fibroproliferative disorder of the palmar fascia ^[9]
• 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 ^[4]
• Nodules progress to form cords in the palmar fascia → flexion contractures ; of the palmar fascia

• The 4^th and 5^th fingers are most commonly involved ^[9]
• Skin puckering near the proximal flexor crease: earliest sign ^[4]
• Palmar nodule
• Palmar cords
• Flexion contracture of affected fingers
• Signs of aggressive disease: knuckle pads (Garrod nodes)
• Plantar fibromatosis (Ledderhose disease): a plantar equivalent of Dupuytren contracture characterized by the development of painless, firm nodules on the sole of the foot. ^[10]
• Peyronie disease

• Usually clinical ^[4]
• Positive "table top test": inability to flatten the palm against the surface of a table due to the contractures in the metacarpophalangeal joints
• Ultrasound of the palm can demonstrate nodules and cords of the palmar fascia
• Fasting blood sugar level

The differential diagnoses listed here are not exhaustive.

• Conservative therapy: Indicated in patients with early disease (skin puckering; nodules) and no functional disability. ^[4]
  • Observation
  • Physiotherapy
  • Hand splint/brace
• Intralesional injections: Indicated in patients with rapidly progressing disease or painful nodules ^[4]
  • Corticosteroids (triamcinolone) ^[13]
  • Collagenase
• Surgery: Indicated in patients with functional disability due to contractures ^[9]
  • Fasciotomy
  • Fasciectomy
• Prognosis
  • Variable prognosis ^[4][9]
  • Recurrence rates are high, even after surgery (∼ 60%) ^[9]