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'''Editors-In-Chief:''' Matthew I. Leibman, M.D.[mailto:MLeibman@partners.org]; Mark R. Belsky, M.D.[mailto:MBelsky@partners.org]; David E. Ruchelsman, M.D.[mailto:DRuchelsman@partners.org]{{KS}}
'''Editors-In-Chief:'''{{Mohamed riad}}, Matthew I. Leibman, M.D.[mailto:MLeibman@partners.org]; Mark R. Belsky, M.D.[mailto:MBelsky@partners.org]; David E. Ruchelsman, M.D.[mailto:DRuchelsman@partners.org]{{KS}}


{{SK}} Dupuytren contracture I, included; DUPC1; palmar fascial fibromatosis; palmar fibromatosis
{{SK}} Dupuytren contracture I, included; DUPC1; palmar fascial fibromatosis; palmar fibromatosis
==Overview==
==Overview==
'''Dupuytren's contracture''' (also known as '''Morbus Dupuytren''') is a fixed [[flexion]] [[contracture]] of the [[hand]] where the [[finger]]s bend towards the palm and cannot be fully extended (straightened). It is named after the famous [[surgery|surgeon]] [[Guillaume Dupuytren|Baron Guillaume Dupuytren]], who described an [[Surgery|operation]] to correct the affliction.
'''Dupuytren's contracture''' (also known as '''Morbus Dupuytren''') is an abnormal [[Thickening of the heart muscle|thickening]] of the hand (palmar fascia) causing curling of [[fingers]] and impaired function of the fingers, especially the little and ring fingers.. It is named after the famous [[surgery|surgeon]] [[Guillaume Dupuytren|Baron Guillaume Dupuytren]], who described an [[Surgery|operation]] to correct the affliction. It is thought that Dupuytren's contracture is the result of [[Microvascular disease|microvascular]] [[angiopathy]]. The incidence of [[Dupuytren's contracture (patient information)|Dupuytren's contracture]] increases with age; the [[median]] age at diagnosis is 50-70 years. [[men]] are affected more often than [[women]]. Common risk factors in the development of [[Dupuytren's contracture (patient information)|Dupuytren's contracture]] include family history, diabetes, antiepileptic medications, liver disease, smoking, alcoholism and manual work. Dupuytren’s disease is [[Progressive angina|progressive]] in nature with no available definitive [[cure]].


The [[ring finger]] and [[little finger]] are the fingers most commonly affected; the [[middle finger]] may be affected in advanced cases, but [[index finger]] and [[thumb]] are nearly always spared. Dupuytren's contracture progresses slowly and is usually [[Pain and nociception|painless]]. In patients with this condition, the [[Biological tissue|tissue]]s under the [[skin]] on the palm of the hand thicken and shorten so that the [[tendon]]s connected to the fingers cannot move freely. The [[palmar aponeurosis]] becomes hyperplastic and undergoes contracture. As a result, the affected fingers start to bend more and more and cannot be straightened.
==Historical Perspective==


[[Incidence]] increases after the [[senescence|age]] of 40; at this age [[men]] are affected more often than [[women]]. After the age of 80 the distribution is about even.
*Dupuytren's contracture was named after [[Guillaume Dupuytren|Baron Guillaume Dupuytren]], a famous surgeon, following describing an [[Surgery|operation]] to correct the affliction.
 
== Historical Perspective ==
 
* Dupuytren's contracture was named after [[Guillaume Dupuytren|Baron Guillaume Dupuytren]], a famous surgeon, following describing an [[Surgery|operation]] to correct the affliction.


==Pathophysiology==
==Pathophysiology==


=== Pathogenesis ===
===Pathogenesis===


*The exact pathogenesis of [[Dupuytren's contracture (patient information)|Dupuytren's contracture]] is not fully understood.
*The exact pathogenesis of [[Dupuytren's contracture (patient information)|Dupuytren's contracture]] is not fully understood.
*It is thought that Dupuytren's contracture is the result of [[Microvascular disease|microvascular]] [[angiopathy]].
*It is thought that Dupuytren's contracture is the result of [[Microvascular disease|microvascular]] [[angiopathy]].


=== Associated Conditions ===
===Associated Conditions===


* [[Diabetes mellitus]]
*[[Diabetes mellitus]]
* [[Thyroid disease|Thyroid diseases]]
*[[Thyroid disease|Thyroid diseases]]
* [[Liver diseases|Liver disease]]
*[[Liver diseases|Liver disease]]
* [[Alcoholism]] and [[smoking]]
*[[Alcoholism]] and [[smoking]]


<br />
==Causes==
==Causes==
The cause of Dupuytren's contracture has not been identified.  
The cause of Dupuytren's contracture has not been identified.  
==Symptoms==
In Dupuytren's disease, the tough connective tissue within one's hand becomes abnormally thick, which can cause the [[finger]]s to curl, and can result in impaired function of the fingers, especially the small and ring fingers.  It usually has a gradual onset, often beginning as a tender lump in the palm.  Over time, pain associated with the condition tends to go away, but tough bands of tissue may develop. These bands, which are the source of the reduced mobility commonly associated with the condition, are visible on the surface of the palm, and may appear similar to a small [[callus]].  It commonly develops in both hands, and has no connection to dominant- or non-dominant hands, nor any correlation with right- or left-handedness.
The contracture sets on very slowly, especially in women.  However, when present in both hands, and when there is associated foot involvement, it tends to accelerate more rapidly.


==Epidemiology and Demographics==
==Epidemiology and Demographics==
Line 59: Line 49:
===Age===
===Age===


*The incidence of [[Dupuytren's contracture (patient information)|Dupuytren's contracture]] increases with age; the [[median]] age at diagnosis is 50-70 years.
*The incidence of [[Dupuytren's contracture (patient information)|Dupuytren's contracture]] increases with age; the [[median]] age at [[diagnosis]] is 50-70 years.


===Race===
===Race===
Line 67: Line 57:
===Gender===
===Gender===


*[[Male|Males]] are more commonly affected by Dupuytren's contracture than [[Female|females]]. The male to female [[ratio]] is approximately 3 to 1.
*[[Male|Males]] are more commonly affected by [[Dupuytren's contracture (patient information)|Dupuytren's contracture]] than [[Female|females]]. The male to female [[ratio]] is approximately 3 to 1.


<br />
==Risk Factors==
==Risk Factors==
Common risk factors in the development of [[Dupuytren's contracture (patient information)|Dupuytren's contracture]] include:
Common risk factors in the development of [[Dupuytren's contracture (patient information)|Dupuytren's contracture]] include:
Line 75: Line 64:
*[[Family history]]: [[genetic predisposition]] may account for 80% of cases.
*[[Family history]]: [[genetic predisposition]] may account for 80% of cases.
*[[Diabetes]] and its medications
*[[Diabetes]] and its medications
*Smoking
*[[Smoking]]
*[[Alcohol]] intake
*[[Alcohol]] intake
*[[Antiepileptics|Antiepileptic]] medications  
*[[Antiepileptics|Antiepileptic]] medications
*There is also some speculation that [[Dupuytren's contracture (patient information)|Dupuytren's contracture]] may be caused by [[physical trauma]], such as manual [[Labor (childbirth)|labor]] or other over-exertion of the hands. However, the fact that Dupuytren's is not connected with handedness casts some doubt on this claim.
*There is also some speculation that [[Dupuytren's contracture (patient information)|Dupuytren's contracture]] may be caused by [[physical trauma]], such as manual [[Labor (childbirth)|labor]] or other over-exertion of the hands. However, the fact that Dupuytren's is not connected with handedness casts some doubt on this claim.
*[[Surgery]] of the hand may trigger growth of Dupuytren nodules and cords if an inclination existed before.
*[[Surgery]] of the [[hand]] may trigger growth of Dupuytren [[Nodule (medicine)|nodules]] and [[Cords of Billroth|cords]] if an inclination existed before.
*Certain occupations with repetitive [[hand trauma]]
*Certain occupations with repetitive [[hand trauma]]


==Natural History, Complications and Prognosis==
==Natural History, Complications and Prognosis==
<br />
 
===Natural History===
 
*Dupuytren’s disease is [[Progressive angina|progressive]] in nature with no available definitive [[cure]].
*Improvement of the [[painful]] [[Nodule (medicine)|nodules]] may occur with time, and many patients never experience a [[contracture]].
 
===Complications===
 
*[[Ectopic]] disease involving the [[penis]] (Pyronie's disease) or the feet ([[Ledderhose's disease|Ledderhose disease]])
*Limitation of daily activities and work capabilities
 
===Prognosis===
 
*[[Prognosis]] is generally poor.
*Development of [[Dupuytren's disease]] at an earlier age is associated with more severe [[Deformity|deformities]].
 
==Diagnosis==
==Diagnosis==
===Diagnostic Study of Choice===
Dupuytren’s disease is primarily diagnosed based on the [[clinical]] presentation.
===History and Symptoms===
*Symptoms of '''Dupuytren's disease''' include abnormal [[Thickening of the heart muscle|thickening]] of the hand ([[palmar fascia]]) causing curling of [[fingers]] and impaired function of the fingers, especially the little and ring fingers.
*It usually has a gradual onset, often beginning as a [[Tenderness (medicine)|tender]] [[lump]] in the [[Palmar|palm]].
*It often starts as a [[Nodule (medicine)|nodule]], usually in line with the [[ring finger]].
*Over time, [[pain]] associated with the condition tends to go away, but tough [[bands]] of tissue may develop.
*These bands, which are the source of the reduced [[mobility]] commonly associated with the condition, are visible on the surface of the [[Palmar|palm]], and may appear similar to a small [[callus]].
*It commonly develops in both hands, and has no connection to [[dominant]]- or non-dominant hands, nor any correlation with right- or left-[[handedness]].
*The [[contracture]] sets on very slowly, especially in women.  However, when present in both hands, and when there is associated [[foot]] involvement, it tends to accelerate more rapidly.
===Physical Examination===
===Physical Examination===
====Skin====
 
=====Hand=====
* The earliest sign of a Dupuytren's contracture is a triangular "puckering" of the [[palmar]] skin over the [[Flexion|flexor]] [[tendon]] just before the flexor crease of the finger, at the [[metacarpophalangeal joint]].
* The most commonly affected finger is the [[Ring finger|ring]] and [[little finger]]. The [[thumb]] and [[Index finger|index]] fingers rarely involved.
<gallery>
<gallery>
File:Dupuytren contracture01.jpg|Dupuytren contracture. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/ Adapted from Dermatology Atlas.]''</SMALL></SMALL>
File:Dupuytren contracture01.jpg|Dupuytren contracture. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/ Adapted from Dermatology Atlas.]''</SMALL></SMALL>
File:Dupuytren contracture02.jpg|Dupuytren contracture. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/ Adapted from Dermatology Atlas.]''</SMALL></SMALL>
File:Dupuytren contracture02.jpg|Dupuytren contracture. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/ Adapted from Dermatology Atlas.]''</SMALL></SMALL>
File:Dupuytren contracture03.jpg|Dupuytren contracture. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/ Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
File:Dupuytren contracture03.jpg|Dupuytren contracture. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/ Adapted from Dermatology Atlas.]''</SMALL></SMALL>
File:Dupuytren contracture04.jpg|Dupuytren contracture. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/ Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
File:Dupuytren contracture04.jpg|Dupuytren contracture. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/ Adapted from Dermatology Atlas.]''</SMALL></SMALL>
File:Dupuytren contracture05.jpg|Dupuytren contracture. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/ Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
File:Dupuytren contracture05.jpg|Dupuytren contracture. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/ Adapted from Dermatology Atlas.]''</SMALL></SMALL>
File:Dupuytren contracture06.jpg|Dupuytren contracture. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/ Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
File:Dupuytren contracture06.jpg|Dupuytren contracture. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/ Adapted from Dermatology Atlas.]''</SMALL></SMALL>
</gallery>
</gallery>
==Treatment==
==Treatment==


*Surgery (in cases of severe contracture removes the contracture)
===Medical Therapy===
*Radiation therapy (specifically in early stages inhibits development of contracture)
*Needle aponeurotomy (releases the contracture)
*[[Triamcinolone]] (kenalog) injections provide some relief


Surgical management consists of opening the skin over the affected cords and excising, removing, the fibrous tissue. The fingers may then be brought out to length with the help of postoperative therapy. The procedure is not curative in that remaining non-affected fascia may still develop Dupuytren's disease later on, and therefore the patients may need repeat surgery. In addition, the thickened fascia often invests the digital nerves and arteries, so there is some risk of nerve and/or arterial injury.
*Pharmacologic medical therapies for Dupuytren's disease include pain management and corticosteroid injection into the nodules,
*In some cases, repeated corticosteroid injections may cause softening of the nodules and slow down the disease progression.
*Most cases with simple nodules and without significant functional impairment benefit from conservative medical therapy.


Treatment of Dupuytren's disease with low energy x-rays (radiotherapy) may cure Morbus Dupuytren on a long term, specifically if applied in early stages of the disease. Needle aponevrotomy is a minimal invasive technique where the cords are weakened through the insertion and manipulation of a small needle. Once weakened, the offending cords may be snapped by simply pulling the finger(s) straight. The nodules are not removed and might start growing again. Currently in phase III of [[Food and Drug Administration]] (FDA) approval is another promising therapy, the injection of [[collagenase]]. This procedure is similar to needle aponevrotomy, however the chords are weakened through the injection of small amounts of an enzyme that dissolves them.
===Interventions===


Dupuytren's contracture is not a dangerous condition, and often no treatment is necessary. Even when treatment is used, there is no permanent way to stop or cure the infliction.  If there is a painful lump present, an injection can often help alleviate the pain.  If pain persists or the function of the hand becomes seriously impaired, individuals often choose to have surgery.  This surgery usually entails removing or separating the thickened bands, and is unlikely to cause any major complications or side effects, although in serious cases, some minor skin grafting may be necessary.  Any damaged skin or tissue in the surgically affected area of the hand will heal fairly quickly, and reducing bloodflow during the healing period helps reduce pain.  This may be done by keeping the hand raised or elevated so that blood does not pool in the affected area.
====Collagenase clostridium histolyticum (CCH) injections====


After surgical treatment, the condition may worsen in inadequately excised diseased tissue or re-develop in previously normal tissue.
*The [[mechanism of action]] is to weaken the [[contracted]] [[cord]] by breaking down [[collagen]].
*It is done in an outpatient practice but requires another clinic visit to snap the cord.
*I t is generally safe; however several [[Complication (medicine)|complications]] were reported, such as [[tendon]] [[rupture]] and pulley injury.
*CCH injectiont is available in only North America after its withdrawal from European, Australian, and Asian markets.
 
===Surgery===
[[Surgery]] is not the first-line treatment option for patients with [[Dupuytren's contracture (patient information)|Dupuytren's]] disease. Surgery is usually reserved for patients with either:
 
*Rapid progression over a few months
*Severe thumb [[Contracture|contractures]] with functional impairment
*More than 30° [[metacarpophalangeal joint]] contracture or 10-20° [[Proximal interphalangeal joints|proximal interphalangeal joint]] [[contracture]]
 
The choice of the type of surgery depends the following variables:
 
*Severity of the disease
*Individual characteristics (such as [[age]], [[Occupational Medicine|occupation]], degree of functional disability)
*Patient and/or physician preference
 
Surgical options include:
 
*'''[[Percutaneous]] [[needle]] [[fasciotomy]] (PNF):''' involves division of the cord using a needle. It is generally effective and safe with no [[Complication (medicine)|complications]]; however [[tendon]] and [[Digital clubbing|digital]] [[nerve]] and [[Blood vessel|vessel]] damage were reported in some cases. Its efficacy is low in the management of extensive [[Contracture|contractures]] or contractures involving the [[Interphalangeal articulations of hand|proximal interphalangeal joints]]. The recurrence rate is high.
*'''[[Fasciectomy]]''' '''either partial (segmental aponeurectomy) or complete (limited fasciectomy):''' involves excision of the cord with higher rate of [[complications]] PNF.
*'''Dermofasciectomy:''' a more [[Invasive (medical)|invasive]] procedure that involves excising the whole diseased tissue, along with the overlying [[subcutaneous fat]] and [[skin]]. It is reserved for more severe cases with extensive skin involvement when the other surgical options have failed. The complication rate is the highest among the three surgical options. The [[Recurrence plot|recurrence]] rate is low.
 
<br />


==External links==
==External links==

Latest revision as of 07:16, 7 August 2021

Dupuytren's contracture
Dupuytren's contracture of the fourth digit (ring finger).
ICD-10 M72.0
ICD-9 728.6
OMIM 126900
DiseasesDB 4011
MedlinePlus 001233

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Editors-In-Chief: Mohamed Riad, M.D.[1], Matthew I. Leibman, M.D.[2]; Mark R. Belsky, M.D.[3]; David E. Ruchelsman, M.D.[4]Kiran Singh, M.D. [5]

Synonyms and keywords: Dupuytren contracture I, included; DUPC1; palmar fascial fibromatosis; palmar fibromatosis

Overview

Dupuytren's contracture (also known as Morbus Dupuytren) is an abnormal thickening of the hand (palmar fascia) causing curling of fingers and impaired function of the fingers, especially the little and ring fingers.. It is named after the famous surgeon Baron Guillaume Dupuytren, who described an operation to correct the affliction. It is thought that Dupuytren's contracture is the result of microvascular angiopathy. The incidence of Dupuytren's contracture increases with age; the median age at diagnosis is 50-70 years. men are affected more often than women. Common risk factors in the development of Dupuytren's contracture include family history, diabetes, antiepileptic medications, liver disease, smoking, alcoholism and manual work. Dupuytren’s disease is progressive in nature with no available definitive cure.

Historical Perspective

Pathophysiology

Pathogenesis

Associated Conditions

Causes

The cause of Dupuytren's contracture has not been identified.

Epidemiology and Demographics

Prevalence

Age

Race

  • Dupuytren's contracture usually affects individuals of the northern Europeans race (Icelandic and Scandinavian populations).

Gender

Risk Factors

Common risk factors in the development of Dupuytren's contracture include:

Natural History, Complications and Prognosis

Natural History

Complications

Prognosis

Diagnosis

Diagnostic Study of Choice

Dupuytren’s disease is primarily diagnosed based on the clinical presentation.

History and Symptoms

  • Symptoms of Dupuytren's disease include abnormal thickening of the hand (palmar fascia) causing curling of fingers and impaired function of the fingers, especially the little and ring fingers.
  • It usually has a gradual onset, often beginning as a tender lump in the palm.
  • It often starts as a nodule, usually in line with the ring finger.
  • Over time, pain associated with the condition tends to go away, but tough bands of tissue may develop.
  • These bands, which are the source of the reduced mobility commonly associated with the condition, are visible on the surface of the palm, and may appear similar to a small callus.
  • It commonly develops in both hands, and has no connection to dominant- or non-dominant hands, nor any correlation with right- or left-handedness.
  • The contracture sets on very slowly, especially in women. However, when present in both hands, and when there is associated foot involvement, it tends to accelerate more rapidly.

Physical Examination

Treatment

Medical Therapy

  • Pharmacologic medical therapies for Dupuytren's disease include pain management and corticosteroid injection into the nodules,
  • In some cases, repeated corticosteroid injections may cause softening of the nodules and slow down the disease progression.
  • Most cases with simple nodules and without significant functional impairment benefit from conservative medical therapy.

Interventions

Collagenase clostridium histolyticum (CCH) injections

  • The mechanism of action is to weaken the contracted cord by breaking down collagen.
  • It is done in an outpatient practice but requires another clinic visit to snap the cord.
  • I t is generally safe; however several complications were reported, such as tendon rupture and pulley injury.
  • CCH injectiont is available in only North America after its withdrawal from European, Australian, and Asian markets.

Surgery

Surgery is not the first-line treatment option for patients with Dupuytren's disease. Surgery is usually reserved for patients with either:

The choice of the type of surgery depends the following variables:

  • Severity of the disease
  • Individual characteristics (such as age, occupation, degree of functional disability)
  • Patient and/or physician preference

Surgical options include:

  • Percutaneous needle fasciotomy (PNF): involves division of the cord using a needle. It is generally effective and safe with no complications; however tendon and digital nerve and vessel damage were reported in some cases. Its efficacy is low in the management of extensive contractures or contractures involving the proximal interphalangeal joints. The recurrence rate is high.
  • Fasciectomy either partial (segmental aponeurectomy) or complete (limited fasciectomy): involves excision of the cord with higher rate of complications PNF.
  • Dermofasciectomy: a more invasive procedure that involves excising the whole diseased tissue, along with the overlying subcutaneous fat and skin. It is reserved for more severe cases with extensive skin involvement when the other surgical options have failed. The complication rate is the highest among the three surgical options. The recurrence rate is low.


External links

http://www.nlm.nih.gov/medlineplus/ency/article/001233.htm

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