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Where Do You Get Dupuytren's Contracture

December 14, 2020

In 1834, the medical peer-reviewed weekly journal, The Lancet, published an article by French anatomist, Guillaume Dupuytren, on his operation of a fibroepithelial condition in which the patient had an abnormal flexion in the fingers furthest away from the thumb. 

Dupuytren was the first to describe the debilitating and disabling condition in clear terms. Before his article, abnormal flexion was rarely (unless it caused pain) considered as an abnormality.

However, due to the 21st century and modern rheumatology, Dupuytren's Contracture has been extensively studied, diagnosed, and several treatments have been suggested for its correction. 

What is Dupuytren's Contracture?

Dupuytren's contracture (du-pwe-TRANZ) is also known as Dupuytren's disease, Morbus Dupuytren, Celtic Hand, and Viking's Disease. This condition is characterized by abnormal thickening in the underlying fascia that manifests as nodules in the overlying skin. This condition is seldom, if ever, painful. Pain is usually a secondary feature to trauma or any other underlying rheumatologic disease (such as rheumatoid arthritis). 

The characteristic feature seen in Dupuytren's Contracture hand is the permanent flexion in fingers away from the thumb. In order of most prevalent to least, the contracture can occur in the; ring finger, little finger, and middle finger. 

The exact etiology of the disease is unknown. However, this condition is seen predominantly in men over the age of fifty with a history of smoking, diabetes, and alcoholism. The disease was previously seen exclusively in people of Nordic origin. 

Etymology: Why is it Called Viking's Disease?

Even before Guillaume Dupuytren's article, the disease had been documented in folklore. The earliest accounts of flexion in the hands after a certain age was documented in folklore and fables from the time of the Vikings. 

The Vikings believed that the disease was caused by a curse - they had theorized that the curling of the fingers (which they deemed as "Hand of Benediction") was a curse on the bagpipe of a man from the MacCrimmon clan. 

Guillaume Dupuytren operated on a hand with the rheumatologic disease in 1831 and published his findings in 1834. The disease would come to be known as his medical discovery. However, a prominent surgeon before Dupuytren's time, Henry Cline, had previously published his own findings on the disease. Albeit Cline could not give many details regarding the flexion besides presenting signs and symptoms. 

Pathophysiology of the Condition

The exact underlying cause of This condition is unknown, but there has been extensiveresearch on the pathophysiology i.e., the mechanism of disease progression in Dupuytren's Disease. 

The disease is fibroproliferative, a collection of the group including keloid scars. The nodule forms as a result of fascia thickening due to unstimulated fibroblast proliferation. The collagen seen is predominantly type III collagen. The myofibroblasts are prominent in Dupuytren's tissue.

Regulation of several growth factors plus receptors have also shown in the tissue, that includes the transforming growth factor, factor β, and fibroblast growth factor. 

Dupuytren's Contracture affects the fascia, specifically the palmar fascia. This fascia is a aponeurotic sheet which underlies inside in the dermis and the overlying to the flexor tendons plus the small muscles in the hand. 

The fascia moves outwards over the wrist through the palm, proceeding as the palmo-digital fascia first. Then as the digital fascia. The fibers then remain longitudinal. As well as protecting deeper structures.

Progression of the Disease

Dupuytren's Contracture hand starts as Dupuytren's Disease. The condition takes several years to manifest into permanent flexion of the fingers. Initially, the flexion can be overturned (i.e., the extension is possible against some resistance) and the patient might feel mild soreness in the affected region that resolves on its own. 

The thickening of the fascia begins as pits and mild swelling and eventually progresses into nodular like masses. The stage wherein the patient notices the nodules is usually where he or she visits a doctor. However, even with the nodules, the patient can still extend his or her fingers. 

Another prominent feature seen simultaneous with the development of the nodules is the curling of the associated fingers. As more time progresses, the nodules tether to one another and the skin causing the curling to worsen. It is at this point that the patient can not extend his or her fingers anymore. 

The soreness is practically non-existent as the disease goes on. 

Diagnosing Dupuytren's Contracture

It is important to understand that the condition manifests itself in a matter of years rather than days. In some documented cases, the condition began as a thickening in the skin in the patient's youth and progressed to the formation of nodules by the time they reached middle age. 

It is also important to understand that this condition is not one of the skin, rather of the fascia underlying the skin. The fascia is a band of fibrous tissue that anchors the skin onto the underlying muscle and bone. The thickening is seen in the fascia which manifests itself on the skin and not the other way around. 

Presentation

A patient can present to a physician's office for a routine checkup or a condition other than Dupuytren's Disease. This condition is mostly diagnosed as part of a routine general physical examination. 

In most cases where Dupuytren's Disease is the presenting complaint, the patient can present to the doctor with a history of either one of two scenarios:

  • Pain in the nodules, or
  • Increase in size (from nodules to cords) and perceived threat of malignancy

The condition begins as pitting or deepening in the palm that eventually progresses into a nodule over the area of the affected finger and in later stages transforms into cords. 

The abnormal and permanent flexion can cause a wide range of complications in the normal functioning of the patient, such as:

  • Trouble putting on clothes
  • Social embarrassment from hand shaking
  • Trouble cooking and cleaning

In a nutshell, the main complaints that the patient has over the condition are regarding day to day functioning. Pain is seldom, if ever, a feature unless there's a secondary cause such as trauma or any other condition.

History Taking

While taking the patient's history, the following factors are notable:

Risk Factors:

  1. Non-Modifiable:
  • Gender; this condition is seen predominantly in males. 
  • Age; this condition is seen in men over the age of fifty. 
  • Ethnicity; men of Scandanavian origin or Northern European heritage are known to have the condition more than in other ethnicities. 
  • Familial history; 60-70% of the cases are reported in patients with a previous family history of the condition. 
  1. Modifiable:
  • Heavy Smoking; seen in smokers who smoke over 25 cigarettes a day.
  • Alcoholics
  • People with lower BMI's
  • People with higher blood glucose levels
  1. Associated Conditions:
  • Diabetes
  • HIV
  • Epilepsy
  • Plantar Fibromatosis
  • Myocardial Infraction

Examination

The 'finger-table' test is one of the most common tests used to diagnose Dupuytren's Contracture. The procedure is simple; ask the patient to lay their hand flat out on the table in front of them. 

Understandably, a patient who's fingers are bent or curled permanently will be unable to lay their fingers flat on the table. 

Types

  • Type I - The most aggressive form of the condition seen in only 3% of patients. Also known as Dupuytren's diathesis. The risk factors are non-modifiable in most cases and associated with other symptoms as well. These other symptoms include knuckle pads and Ledderhose Disease. 
  • Type II - Normal or moderate type of Dupuytren's Disease found solely in the palm and beginning after the age of fifty in patients. The condition may be aggravated by secondary and other conditions in conjecture to Dupuytren's Disease
  • Type III - The mildest form of the disease is seen in people with compromised immune systems, diabetics, and people who are HIV positive or with epilepsy. The disease is a manifestation of other conditions and the abnormal flexion thus can be overturned in most cases (albeit against resistance).

Treatment

Treatment options are either surgical or non-surgical:

Non-Surgical Treatment Options:

  • Steroid Injections - Corticosteroid injections are indicated for pain, to reduce swelling, and when there is soreness in the nodule. 
  • Splinting - This non-surgical treatment usually precedes surgical treatment. 
  • Radiation therapy.
  • Alternative Medicine

Surgical Treatment Options:

Less Invasive Procedures:

  • Percutaneous Needle Fasciotomy
  • Extensive Percutaneous Aponeurotomy and Lipografting
  • Collagenase Treatment

Highly Invasive Procedures:

  • Limited Fasciectomy
  • Wide-Awake Fasciectomy
  • Dermofasciectomy
  • Segmental Fasciectomy

Conclusion

Dupuytren's Contracture is a progression in Dupuytren's Disease - a disease of rheumatologic significance involving thickening in the underlying fascia of the hand. This condition is debilitating and disabling involving permanent flexion of the fingers farthest from the thumb. The most commonly involved finger is the ring finger. 

This condition is of unknown etiology, however, there has been extensive research done on the pathophysiology. The only known non-modifiable risk factors of the condition implicate men over the age of fifty with Scandanavian or Northern European lineages. 

Dupuytren's Contracture may manifest as either one of three types, with type II being the most prevalent. This condition is seen in patients during routine examinations, mostly. However, the condition might be diagnosed if the patient presents with mild soreness, nodular swelling, and the perceived notion of malignancy. 


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