What causes Dupuytren's Contracture, characterized by the formation of nodules and thickened tissue in the palmar fascia?

Causes of Dupuytren's Contracture

Dupuytren's contracture is primarily caused by genetic factors, with environmental triggers such as trauma, diabetes, and alcohol use serving as important contributing factors in genetically predisposed individuals. 1

Pathophysiology

Dupuytren's contracture is a progressive fibro-proliferative disease affecting the palmar fascia, characterized by:

• Formation of nodules and thickened tissue in the palmar fascia
• Development of cords that contract over time
• Progressive flexion contractures of the fingers, particularly affecting the ring and little fingers
• Presence of inflammatory nodules in the metacarpal region

Primary Causes

1. Genetic Predisposition:

   • Autosomal dominant inheritance with variable penetrance in approximately 40% of cases 2
   • Higher prevalence in people of Northern European descent 3
   • Some cases show spontaneous and recessive inheritance patterns 2

2. Environmental Factors and Associated Conditions:

   • Trauma and Surgery: Local trauma or previous hand surgery can trigger the condition 4
   • Diabetes mellitus: Strong association with development of the disease 1, 2
   • Alcohol consumption: Regular alcohol use increases risk 2, 3
   • Smoking: Tobacco use is a significant risk factor 3
   • Age and Gender: More common in men over 40 years of age 3

Clinical Presentation and Diagnosis

Dupuytren's contracture is diagnosed clinically based on:

• Small, pitted nodules on the palm (initial presentation)
• Progressive development of thickened cords
• Flexion contractures of the fingers, particularly the ring and little fingers
• Skin tethering and subcutaneous thickening
• Physical examination findings such as palpable cords and nodules 2

Distinguishing Features

It's important to distinguish typical Dupuytren's disease from "Non-Dupuytren's palmar fascial disease," which:

• Is often unilateral (vs. bilateral in classic Dupuytren's)
• Lacks family history
• Is frequently associated with prior ipsilateral hand trauma or surgery
• Shows a non-progressive or partially regressive course
• Develops more rapidly after initial insult (average 3.6 months) 4

Natural Course

The natural course of Dupuytren's contracture varies:

• Can range from relatively benign to aggressive progression
• Most commonly follows a fluctuating course
• Will spontaneously regress without treatment in approximately 10% of cases 3
• Is not curable, but symptoms can be managed 5

Key Points for Clinicians

• X-rays are not diagnostic for Dupuytren's contracture but may be ordered to rule out other conditions
• Early identification allows for monitoring and timely intervention
• Treatment options range from observation to minimally invasive procedures to surgical intervention
• Patients with risk factors should be monitored for early signs of the condition

Understanding the multifactorial etiology of Dupuytren's contracture helps guide appropriate management strategies and patient education about this progressive condition.