Dupuytren’s disease

Dupuytren’s contracture is a disease caracterised by a hypertrophic and dysplasic fibrosis of the superficial palmar aponeurosis of the hand, forming nodules and cords and resulting in invinsible flexion of the fingers. It affects several millions of persons in Europe and North America, leading to hand dishability. Several forms are described as well as the association with Knuckle pads, Ledderhose and Lapeyronie’s disease. Familial history of Dupuytren and diabetes are the most important risk factors.

Needle aponeurotomy is a non surgical, ambulatory treatment wich relies on percutanous section of Dupuytren’s cords under local anaesthesia. This technique allows early treatment with results similar to surgical methods in terms of degree of contracture for Tubiana stage 1 to 3. The low rate of complications and the rapidity of recovery have made of needle aponeurotomy in experienced hands a first line treatment.

 

In 1831 Baron Guillaume Dupuytren, surgeon at the Hôtel Dieu of Paris, described an affection touching the hand characterised by a fibrous retraction of the middle part of the superficial palmar aponeurosis resulting in progressive invincible flexion of the fingers1. In fact, the disease was recognised since the Middle Ages. The Okney saga tells the story of a Danish baron whose hooked finger found miraculous cure... after falling during a pilgrimage. Today, Dupuytren’s contracture affects several millions of individuals in Europe and North America2. The disease usually appears in the late forties, with a sex ratio of eight men for one woman. Apart from familial history (its genetic transmission dates back to the Vikings3) and diabetes both appearing as major risk factors; alcohol abuse, smoking, medications such as phenobarbital or isoniasid, and mechanical stress (ie face climbing or hand labour) are suspected to enhance the occurrence of the contracture. The treatment of Dupuytren’s contracture remained surgical until the middle of the last century. Medical treatments were suggested, such as colchicines, verapamil or intra nodular injections of corticosteroids aiming at the atrophy of the fibrosis but showed little effect. The spectacular results of needle aponeurotomy and diffusion of the technique from France to the rest of Europe and North America during the past decades has deeply improved treatment of Dupuytren’s contracture.

Physiopathology

In Dupuytren’s contracture the superficial foil of palmar aponeurosis undergoes a hypertrophic and dysplasic transformation. Fibroblast multiply, thereby forming nodules and cords, hooking themselves on fibrous structures: transverse carpal ligament, pulley, scars, skin and bones. The fibroblasts acquire retractile power, transforming into myofibroblasts, leading to skin retraction and finger contracture4,5. Tension of the cord enhances the retraction. On a molecular focus, authors have reported an increase in immature collagen fibrils.  Differential expressions of matrix metalloproteinases and excess in oxygen free radicals or TGF beta have also been noticed6-9.

Forms and staging

Several clinical forms of the disease can be found: palmar, palmodigital and strictly digital. These particular forms are more difficult to treat10. In young patients, contracture is especially rapidly evolutive11. Natatory forms implicate the superficial transverse intermetacarpal ligament witch prevents separation of fingers. In derm-adhesive forms skin-wells and skin retractions are frequent. Staging of the deformations relies on Tubiana scale witch counts four stages, depending on the global flexion of the finger: stage 1 from 0 to 45°; stage 2 from 45° to 90°; stage 3 from 30° to 135° and stage 4 over 135° 12.