Dupuytren’s disease (DD) is a benign, generally painless fibroproliferative disorder of the palmar and digital fascia, whereby a thick collagen cord develops, causing flexion deformity -- Dupuytren's contracture (DC) -- of the affected metacarpophalangeal (MP) or proximal interphalangeal (PIP) joints. The pathogenesis of DD is largely genetic, and the complexities of its underlying cellular and subcellular mechanisms are only starting to be understood. Surgery has been the mainstay of treatment for centuries; however, less invasive approaches have been shown to be effective in more recent clinical trials and in clinical practice.
Related anatomy
- Normal anatomy
- Palmar fascia: triangular-shaped structure composed of broad sheets or narrow bands of fibers oriented longitudinally and transversely
- Pretendinous Bands: longitudinal fiber bands running superficial to the flexor tendons from the level of the superficial arch to the MP joint area distally
- Natatory ligament: oriented transversely and lies just beneath the skin at each commissure; sends fibers distally along the lateral border of each digit to merge with the lateral digital sheet (LDS)
- Lateral Digital Sheet (LDS): Formed by merging fibers of the spiral band and natatory ligament; runs lateral to and along side the neurovascular bundle (NVB)
- Grayson ligament: Palmar to the NVB, passing from the flexor sheath to the skin; in DC, it can become part of a lateral cord when it joins the diseased lateral digital sheath (LDS)
- Cleland ligament: Dorsal to the NVB and arises from the phalanges; relatively uninvolved in DC
- Diseased fascia (collagen cords)
- Nodules usually appear before contractile cords
- Central cord: involvement of the pretendinous bands; usually results in metacarpophalangeal joint contracture
- Spiral cord: involvement of pretendinous band, spiral band, LDS, Grayson ligament; generally results in PIP joint contracture
- Natatory cord: develops from the distal fibers of the natatory ligament, resulting in a web-space contracture
- Retrovascular cord: dorsal to NVB; usual cause of a distal interphalangeal (DIP) joint contracture
- Transverse Ligament of Palmar Aponeurosis (TLPA): superficial intermetacarpal ligaments or Skoog's ligament is an important part of the palmar aponeurosis. Radially, the TLPA forms the proximal commissural ligaments. The deep surface of the TLPA attaches to the septa of Legueu and Juvara. This septa forms confluence with the palmar plate, inter-palmar plate ligament, sagittal band and A1 pulley.
Relevant basic science
Luck's Three phases of Dupuytren's Disease
- proliferative (random proliferation of myofibroblasts)
- involutional (myofibroblasts align along tension lines in the palm)
- residual (acellular disease with collagen-laden tissue)
Normal palmar tissue
- type I collagen
- fibroblasts
Dupuytren’s disease (DD)
- Type III collagen predominates
- Myofibroblasts containing bundles of actin microfilaments, conferring contractile properties that fibroblasts do not have.
- Increasing levels of mechanical tension have also been shown to influence fibroblast differentiation into myofibroblasts.
- Adjacent myofibroblasts connect to each other and to collagen via fibronectin.
- Transforming growth factor-beta 1 (TGF-β1), TGF- β2, epidermal growth factor, platelet-derived growth factor and connective tissue growth factor (PDGF) have been suggested to play a role in initiating abnormal cellular proliferation. These growth factors found in Dupuytren’s fascia in abnormal amounts.
- Bone morphogenic protein 4 (BMP-4) is expressed by normal palmar fascia but not by the diseased fascia in reverse-transcription polymerase chain reaction studies. Therefore BMP-4 may play a protective role in normal palmar fascia.
- Hypoxanthine is a byproduct of ischemia found in higher-than-normal levels in Dupuytren's tissue. This free radical may play role in triggering the change of fibroblasts to myofibroblasts in elderly hands that have ischemia from diabetes or peripheral vascular disease.
Incidence and related conditions
- Most prevalent in older men of northern European descent
- Global incidence estimates are 1-3% of Caucasians and increases with advancing age
- Mendelian autosomal dominant inheritance with variable penetrance in 10-30% of cases
- The tenascin C (TNC) gene, associated with fibrotic disease and cell migration, is up regulated in DD
- Often occurs with other fibroproliferative disorders (Ledderhose disease, Peyronie’s disease, knuckle pads), which are associated with Dupuytren diathesis.
Differential diagnosis
- For nodules: callus, ganglion, epithelial inclusion cyst
- For contractures: chronic locked trigger fingers, ulnar nerve palsy with sign of benediction, limited joint mobility (LJM), posterior interosseous nerve palsy or Vaughan-Jackson Syndrome (ruptured extensor tendons)
Note: Limited Joint Mobility (LJM): is seen in 30-50% of type I diabetics and in 25–30% of type II diabetics. LJM causes painless flexion contractures of the PIP and/or DIP joints with pathologic cords. The ring and small fingers are most often affected. No treatment will reliably eliminate these contractures.