Dupuytren disease: pathobiology, cord anatomy and risk factors

1. The contractile cell of the Dupuytren nodule is the myofibroblast, a fibroblast that has acquired a smooth-muscle-like fibrillar system.

True

Gabbiani and Majno's electron-microscopy study of Dupuytren and Ledderhose nodules showed cells with a fibrillar system similar to that of smooth muscle, nuclear deformations typical of contracted cells, and cell-to-cell and cell-to-stroma attachments. They proposed that these fibroblasts have modulated into contractile myofibroblasts whose contraction contributes to the clinical contracture.

• Gabbiani G, Majno G. Dupuytren's contracture: fibroblast contraction? An ultrastructural study. Am J Pathol. 1972;66(1):131-46. PMID 5009249

2. A spiral cord displaces the digital neurovascular bundle towards the midline of the finger and closer to the skin, placing it at risk during fasciectomy.

True

The bundle spirals when diseased fascia superficial to it is connected to disease deep to it — classically when a pretendinous cord connects with the lateral digital sheet and Grayson's ligament. As the cord contracts it draws the bundle towards or beyond the midline and superficially, so careful dissection in this zone is needed to avoid iatrogenic digital neurovascular injury. Hettiaratchy also describes distal and double ('corkscrew') spirals.

• Macey ARM, Thomas R. "The Serpentine Zone": A Surgeon's Guide to the Surface Anatomy of the Digital Neurovascular Spiral in Dupuytren's Contracture. J Hand Microsurg. 2017;10(1):54-56. PMID 29706740
• Hettiaratchy S, Tonkin MA, Edmunds IA. Spiralling of the neurovascular bundle in Dupuytren's disease. J Hand Surg Eur Vol. 2009;35(2):103-8. PMID 19828565

3. Once age is accounted for, pooled data show no association between diabetes mellitus and Dupuytren disease.

False

Broekstra's meta-analysis of 32 studies found an age-adjusted odds ratio of 3.06 (95% CI 2.69–3.48) for Dupuytren disease in people with diabetes. The association appeared stronger for type 1 than for type 2 diabetes, although that difference was not statistically significant; liver disease and epilepsy were also associated with Dupuytren disease.

• Broekstra DC, Groen H, Molenkamp S, Werker PMN, van den Heuvel ER. A Systematic Review and Meta-Analysis on the Strength and Consistency of the Associations between Dupuytren Disease and Diabetes Mellitus, Liver Disease, and Epilepsy. Plast Reconstr Surg. 2018;141(3):367e-379e. PMID 29481401

4. Dupuytren disease predominantly affects the radial side of the hand, and the index finger is the digit most frequently involved.

False

In Lanting's examination of 344 primarily affected hands, the ring finger was the digit most frequently affected and the rays on the ulnar side of the hand predominated. Disease in the thumb and index finger correlated with each other, but there was no evidence of correlation between the radial and ulnar sides of the hand.

• Lanting R, Nooraee N, Werker PMN, van den Heuvel ER. Patterns of Dupuytren disease in fingers: studying correlations with a multivariate ordinal logit model. Plast Reconstr Surg. 2014;134(3):483-490. PMID 24804640

5. Genome-wide association studies have linked susceptibility to Dupuytren disease with genes of the Wnt-signalling pathway.

True

Dolmans's genome-wide association study of 2,325 patients and 11,562 controls identified nine susceptibility loci, six of which contain Wnt-pathway genes, among them WNT4, SFRP4, WNT2 and WNT7B. The authors concluded that aberrations in Wnt signalling are key to the fibromatosis.

• Dolmans GH, Werker PM, Hennies HC, Furniss D, Festen EA, Franke L, et al.. Wnt signaling and Dupuytren's disease. N Engl J Med. 2011;365(4):307-17. PMID 21732829