Dupuytren's disease (Morbus Dupuytren or Dupuytren's contracture)

 

Dupuytren's contracture, also called Morbus Dupuytren (MD), is a benign thickening of the palm's deep connective tissue (fascia). Often the tumor is located in the flexible area of the skin in the vicinity of the base joints of the fingers. Tumors that start in the lower palm, further towards the wrist, an area known as the palmar aponeurosis, are typical less critical and do not lead to contraction. The disease is typically named Dupuytren's contracture, the term Dupuytren's disease might be a bettter term because it includes the initial stage of the disease before the contracture has started.

Dupuytren's contracture, or rather Dupuytren's disease, typically starts on the palm of the hand with a small  nodule or several nodules that can be felt (palpated) and are initially not very hard. There is indication that the disease actually starts earlier with changes in the tissue underlying the skin. Because those changes are on a microscopic level, they generally are not detected and nodules are typically the first indication of Dupuytren disease.

The next stage is the development of cords along tendons. In most cases the fourth or fifth finger is affected first but nodules can also appear throughout the palm, sometimes in the digits, and in rare cases even elsewhere. Typically Dupuytren's disease tends to stabilize temporarily, but the nodules and cords start growing again by degrees over several years. Eventually, as the cords thicken, the contractive forces increase and bend the affected fingers towards the palm (there is actually some indication that there is no real contraction but that the relaxed, bent finger position is fixed by the cord and thus creates an extension deficit. The effect is the same: the finger can't be stretched anymore, resulting in Dupuytren's contracture). A more detailed description of the growth process and the related pathophysiology of Dupuytren's contracture can be found in e-medicine-Revis.

Example of Dupuytren's and Ledderhose nodules at hand and foot, respectively.

Early stage (stage N of Dupuytrens), no finger contraction

and Ledderhose nodules still relatively small.

(Picture provided by Alfried Krupp Hospital, Essen, Germany)

Below is an example of Dupuytren's contracture in a progressed stage. The finger has been bent permanently into the palm. The skin at the contracted joint thus hasn't been sufficiently exposed to air anymore and thus has not been kept sufficiently dry and clean. This had already caused skin damage. After treatment (in this case with NA) the finger became straight again and the skin recovered.

Dupuytren's contracture in progressed stage with already damaged skin.

(Picture provided by A. Meinel, Dupuytren Ambulanz)

What is causing Dupuytren's disease?

Though more than 175 years have passed since 1832 when Baron Guillaume Dupuytren (1777-1835) presented his findings in Paris on the disease that acquired his name, the root causes still remain unknown. There is evidence that an inclination (predisposition) for Dupuytren's contracture can be inherited (S. Hindocha et al. "The heritability of Dupuytren's disease" J Hand Surg [Am]. 2006 Feb;31(2):204-10 abstract). Some additional environmental effect might be required to start the disease (HA Lyall "Dupuytren's disease in identical twins" J Hand Surg [Br]. 1993 Jun;18(3):368-70 abstract). Damage (or rather healing) of the hand can trigger Dupuytren's disease (see also Dupuytren's work related?). There is indication that Dupuytren's contracture is a chronic inflammatory disease.

Oxidative stress in combination with microvessel ischemia (local restriction in blood supply) might be a mechanism driving the onset of Dupuytren's disease, see GA Murrel, FJ Francis, and L Bromley "Free radicals and Dupuytren's contracture" Br Med J (Clin Res Ed) 295 (1987) p 1373 - 5 abstract and IS Yi, G Johnson, and MS Moneim "Etiology of Dupuytren's disease" Hand Clin 15 (1999) p 43 - 51 abstract .

Tension in the palm (palmar fascia) has also been proposed as a factor causing Dupuytren's disease. In a small trial 27 patients had surgical fasciotomy with 13 having a Z-plasty cut reducing post-Op tension. After 2 years 2 patients of that group (15 %) exhibited recurrence while 7/14 (50 %) of the other group, where only a simple transversal cut was used, exhibited recurrence. This might confirm the effect of tension. N. Citron and A. Hearnden "Skin tension in the aetiology of dupuytren's disease; a prospective trial" The Journal of Hand Surgery: Journal of the British Society for Surgery of the Hand 28 (2003) p 528-530 abstract.

Recently it was claimed that the dominant gene for Dupuytren's contracture has been located on the genome (F. Z. Hu et al. "Mapping of an autosomal dominant gene for Dupuytren's contracture to chromosome 16q in a Swedish family" Clinical Genetics 68/5 (2005) p 424 Dupuytren's_genome). Though the gene itself has not yet been identified, this might eventually lead to better therapies for Dupuytren's sometimes in the future (the gene MafB might be involved, see abstract). However, even without knowing the genes involved, we already know much more about the symptoms of the disease and we have made some progress in treating it.

 

Ledderhose's disease is similar to Dupyutren's contracture  except that it affects the feet, typically starting with nodules in the arch (plantar aponeurosis).

A third version of this disease affects the male genital (Peyronie's disease, induratio penis plastica, IPP).

All three diseases are assumed to have the same or similar root causes.

Continued:

Age distribution and prevalence in various countries

Stages and therapies of Dupuytren's disease

Dupuytren's contracture and trauma

Literature on Dupuytren's disease

Page last modified: 04/28/2008