• Clinical science

Dupuytren's contracture (Palmar fibromatosis)

Abstract

Dupuytren's contracture is a common fibroproliferative disorder affecting the palmar fascia mainly of the 4th, and 5th 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 metacarpophalengeal (MCP) and proximal interphalengeal (PIP) joints. Patients with Dupuytren's contracture may also have fibroproliferative disorders at other sites (e.g., plantar fibromatosis and Peyronie's 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 10% of patients. Regardless of the treatment options, recurrence rates are high (∼ 60%).

Epidemiology

  • Prevalence: 4–6%
  • Peak incidence: 40–60 years
  • Sex: >

References:[1]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

  • The exact etiology is unknown
  • Genetic predisposition
  • Risk factors: these factors may cause ischemic injury of the palmar fascia → development of Dupuytren's contracture in genetically predisposed individuals
  • Autoimmunity

References:[2][3][1]

Pathophysiology

References:[2][1][4]

Clinical features

  • The 4th and 5th fingers are most commonly involved
  • Skin puckering near the proximal flexor crease: earliest sign
  • Palmar nodule
  • Palmar cords
  • Flexion contracture of affected finger/s
  • Signs of aggressive disease: Knuckle pads (Garrod nodes) ; Plantar fibromatosis(Ledderhose disease)
  • Peyronie's disease

References:[2][1][5][4]

Diagnostics

  • Usually clinical
  • Ultrasound; of the palm can demonstrate nodules and cords of the palmar fascia
  • Fasting blood sugar level

References:[2][1]

Differential diagnoses

Differential diagnosis of Dupuytren's contracture

Condition Etiology Clinical features

Palmar fasciitis

  • All fingers bilaterally affected (more extensive than in Dupuytren's contracture)
  • Progressive flexion contractures
Claw hand deformity
  • Extension of the MCP with PIP and DIP flexion
  • 4th and 5th fingers affected
  • Numbness of the ulnar aspect of the palm

Stenosing tenosynovitis

(Trigger finger)

  • Painful locking of a finger in flexed position; releases suddenly with a snap/pop on extension
  • A tender nodule is often palpable at the base of the metacarpophalangeal joint
  • Mostly affects thumbs and ring fingers

References:[6]

The differential diagnoses listed here are not exhaustive.

Treatment

The aims of treatment are to arrest the progression of the disease, correct the deformities, and restore joint functionality

  • Conservative therapy: Indicated in patients with early disease (skin puckering; nodules) and no functional disability.
    • Observation
    • Physiotherapy
    • Hand splint/brace
  • Intralesional injections: Indicated in patients with rapidly progressing disease or painful nodules
  • Surgery: Indicated in patients with functional disability due to contractures (any contracture of the PIP, severe MCP contracture).
    • Fasciotomy
    • Fasciectomy
    • Amputation
  • Prognosis
    • Variable prognosis
    • Recurrence rates are high, even after surgery (∼ 60%)

References:[2][1][4]