Dupuytren's disease (also known as Dupuytren's contracture or morbus Dupuytren), is a fixed flexion contracture of the hand due to a palmar fibromatosis, where the fingers bend towards the palm and cannot be fully extended (straightened). It is an inherited proliferative connective tissue disorder which involves the palmar fascia of the hand. It is named after Baron Guillaume Dupuytren, the surgeon who described an operation to correct the affliction in the Lancet in 1831.
The ring finger and little finger are the fingers most commonly affected. The middle finger may be affected in advanced cases, but the index finger and the thumb are nearly always spared.Dupuytren's contracture progresses slowly and is usually painless. In patients with this condition, the palmar fascia thickens and shortens so that the tendons connected to the fingers cannot move freely. The palmar fascia becomes hyperplastic and contracts.
In Dupuytren's contracture, the palmar fascia within the hand becomes abnormally thick, which can cause the fingers to curl and can result in impaired function of the fingers. The small and ring fingers are especially affected. The main function of the palmar fascia is to increase grip of the hand; thus, over time, Dupuytren's contracture decreases patients' ability to grip objects. Pain is usually not associated with this condition. However, substance P nerve fibers positively correlate to the 12-fold increase in mast cells in patients with Dupuytren's contractions, and those patients report pain along with the contractions.
Dupuytren's disease often starts with nodules in the palm of the hand and it can extend to a cord in the finger. The palmar fascia becomes abnormally thick due to the fact that there is a change of collagen type. Normally, the palmar fascia consists of collagen type I, but if a patient has Dupuytren’s disease, the collagen type I changes to collagen type III, which is significantly thicker than collagen type I. The contracture sets in slowly and treatment is indicated when the so-called table top test is positive. With this test, the patient places his hand on a table. If the hand lies completely flat on the table, the test is considered negative. If the hand cannot be placed completely flat on the table, but there is a space between the table and a part of the hand as big as the diameter of a ballpoint pen, the test is considered positive and surgery or other treatment may be indicated. Additionally, finger joints may become fixed and rigid. Also other components of the extracellular matrix are involved, including fibronectin, proteoglycan-4, etc.
Down-regulation of collagen synthesis and matrix metalloproteinase expression in myofibroblasts from dupuytren nodule using adenovirus-mediated relaxin gene therapy
The pathobiochemistry of Dupuytren's contracture
Expression of VEGF, its receptors, and HIF-1α in Dupuytren's disease
Incidence increases after the age of 40; at this age, men are affected more often than women. After the age of 80, the gender distribution is about even.
Dupuytren's disease has been referred to as a Viking or Celtic disease, but existed in Europe earlier than the Viking Age and originated much earlier in prehistory.
Dupuytren's contracture is a non-specific affliction, but primarily affects:
- People of Scandinavian or Northern European origins, though it is also widespread in some Mediterranean countries and in Japan.
- People with a family history (60% to 70% of those afflicted have a genetic predisposition to Dupuytren's contracture).
- People affected by liver cirrhosis.
- Rock climbers
- People affected by physical trauma such as manual labor or other over-exertion of the hands.
Association among work exposure, alcohol intake, smoking and Dupuytren's disease in a large cohort study
Treatment for Dupuytren's disease might involve one or more different types of treatments with some hands needing repeated treatments.
The main categories listed by the International Dupuytren Society in order of stage of disease are Radiation Therapy, Needle Aponeurotomy(NA), Collagenase Injection (Xiaflex) and Hand Surgery.
Efficacy and safety of concurrent collagenase clostridium histolyticum injections for multiple dupuytren contractures
Radiotherapy in early-stage Dupuytren's contracture. Long-term results after 13 years
Knowledge about Dupuytren's syndrome has improved in particular in the last two decades thanks to the new technologies. The increase of the scientific background will help to develop new therapies. Some of the new alternative therapies have already been discussed, but they haven't been approved.