Dupuytren's Disease

Dupuytren's Disease: Intro & Risk Factors - Puckering Palms Primer

• Benign, progressive fibroproliferative disorder of palmar/digital fascia.
• Forms nodules, cords → flexion contractures (MCP, PIP joints). Ulnar digits (ring, little) common.
• Risk Factors:
  • Age (>50 yrs), Male, Family Hx, Northern European descent.
  • 📌 DDAShT (Mnemonic: Diabetics Drink Alcohol & Smoke Tobacco):
    • Diabetes, Dyslipidemia
    • Alcohol
    • Smoking
    • Hepatic disease, HIV
    • Trauma, anti-epilepTics

⭐ Most common in males of Northern European descent, often bilateral.

Dupuytren's Disease: Pathoanatomy & Genetics - Fibrous Finger Fiasco

• Pathoanatomy: Fibroproliferative disorder of palmar/digital fascia.
  • Key cells: Myofibroblasts (α-SMA+).
  • Collagen: ↑ Type III, ↓ Type I.
  • Affected structures:
    • Prebendinous bands → cords.
    • Natatory ligaments → web space contractures.
    • Spiral cord (of Gosset) → PIP joint contracture; displaces neurovascular bundle (NVB) superficially & medially.
    • Lateral digital sheet.
    • Grayson's & Cleland's ligaments (less common).
• Genetics:
  • Autosomal dominant inheritance, variable penetrance.
  • Strong association: Northern European descent ("Viking disease").
  • Possible gene links: Wnt signaling pathway (e.g., WNT2, WNT7B).

⭐ Pathologically characterized by proliferation of myofibroblasts and deposition of Type III collagen replacing Type I collagen.

Dupuytren's Disease: Clinical Features & Staging - Contracture Clues & Count

• Features:
  • Palmar/digital nodules → cords.
  • Skin: Pitting, tethering.
  • Contractures: MCPJ, PIPJ (ring, little finger common).
  • Garrod's pads (dorsal PIPJ, ~50%).
• Hueston's Tabletop Test: Palm flat on table? Positive if unable (MCPJ contracture).

  ⭐ Positive Hueston's test: MCPJ contracture. Surgery: MCPJ >30° or any PIPJ contracture.

• Staging (Tubiana): Total flexion deformity (MCPJ+PIPJ+DIPJ).

  Stage	Deformity (°)
  0	No lesion
  N	Nodule only
  1	1-45
  2	46-90
  3	91-135
  4	>135

Dupuytren's Disease: Management Spectrum - Releasing the Grip

• Conservative Management:

  • Observation: Mild disease, no functional loss.
  • Splinting: Generally ineffective for preventing progression.
  • Steroid injections: May soften nodules, limited long-term benefit.

• Minimally Invasive Options:

  • Collagenase Clostridium Histolyticum (CCH) Injection: Enzymatic fasciotomy. For palpable cords, MCPJ/PIPJ contractures.
  • Needle Aponeurotomy (NA): Percutaneous cord disruption. Best for single, well-defined MCPJ cords.

• Surgical Management: Indications: MCPJ contracture >30°, any PIPJ contracture, or significant functional impairment.

  • Fasciectomy:
    • Limited/Segmental: Excision of diseased fascia. Most common.
    • Radical/Total: Excision of all palmar fascia (↑morbidity, less common).
    • Dermofasciectomy: Excision of fascia + overlying skin, then skin graft. For severe/recurrent disease.
  • Salvage: Amputation (rare, severe recurrent cases).

• Post-Intervention Care:

  • Splinting (night-time, extension).
  • Hand therapy: Crucial for ROM and function.

⭐ Needle aponeurotomy is suitable for mild, single-cord MCPJ contractures but has a higher recurrence rate than fasciectomy.

High‑Yield Points - ⚡ Biggest Takeaways

• Progressive fibroproliferative disorder of the palmar and digital fascia.
• Strong links: Northern European descent, diabetes mellitus, alcoholism, smoking.
• Features painless palmar nodules and cords, causing progressive flexion contractures.
• Primarily affects the ring and little fingers (ulnar side).
• Hueston's tabletop test is positive (patient cannot lay hand flat on table).
• Surgery (e.g., fasciectomy) for MCP contracture >30° or any PIP joint contracture.
• Myofibroblasts are the key pathological cells involved in contracture.