Superficial fibromatosis that starts in the hand

Aetiology

• Genetic predisposition
  • Autosomal dominant mutation with variable penetration
  • Common in northern Europe
  • Higher incidence in males
• Environmental factors
  • Diabetes mellitus
  • Alcohol/cirrhosis
  • Smoking
  • Epilepsy/epileptic medication
  • Repetitive trauma or from an acute injury to the hand

Pathophysiology

• Excessive myofibroblast proliferation and altered collagen matrix composition leads to thickened and contracted palpar fascia
• The thickening and contracture of the subdermal fascia leads to fixed flexion deformity of fingers (NOT associated with a tendon)
• Bands are primarily collagen type III

Clinical features

Symptoms

• Painless, gradual progression
• Usually starts as palmar pit/nodule
• Flexion contracture of affected fingers, 4th and 5th fingers are the most commonly involved
• Dupuytren’s diathesis - severe form of Dupuytren’s involving little and ring fingers, Lederhosen's (superficial fibromatosis of the foot) and Peyronie's (superficial fibromatosis of the penis)

Signs

• Palpate cords
• MCP/PIP joint involvement - measure angles
• Table-top test - inability to flatten the palm against the surface of a table due to the contractures in the metacarpophalangeal joints

Investigations