Dupuytren's contracture is a progressive disorder that affects the palmar fascia, causing the fibrous tissue to shorten and thicken.
Excessive myofibroblast proliferation and altered collagen matrix composition lead to thickened and contracted palmar fascia. The resultant digital flexion contractures may severely limit function.
- Typically, it affects Northern European men (especially from Scotland, Iceland and Norway). In Northern European men over the age of 60, there is 25% prevalence reported.
- It is up to six times more common in men than in women.
- Onset is usually in the mid-50s. (In women onset tends to be in the mid-60s.)
- 3-5% of the population in the UK are affected.
- It may affect as many as 2 million people in the UK but only a small number will require surgery.
- It is one of the most common conditions seen by hand surgeons.
- Autosomal dominant inheritance of varied penetrance is suggested.
The cause is unknown but genetic predisposition, trauma, inflammatory response, ischaemia, environmental factors and variable expression of proteins and growth factors within the local tissue have been implicated in the disease process. There is increasing evidence of a strong genetic component.
- Increasing age.
- North European descent.
- Positive family history.
- Smoking. (Reported to be three times more common in smokers.)
- Alcohol excess (but most affected people do not have alcohol dependence).
- Diabetes mellitus. (One in five people with diabetes are reported to develop Dupuytren's contracture, albeit a less severe form. It is equally common in types 1 and 2 but develops at a younger age in people with type 1 diabetes.)
- Occupational exposure to hand-transmitted vibration. (A report of the evidence by the Industrial Injuries Advisory Council in 2014 concluded that significant exposure to hand-held vibrating tools can more than double the risk.) Heavy manual work without vibration exposure may also increase the risk.
- Raised lipid levels.
- Epilepsy/anticonvulsant drugs/epilepsy. (It is not clear whether the association is with epilepsy or the medication used to control it.)
- Most commonly, it affects the ring finger, followed by the little finger and then the middle finger. It can affect any digit.
- It is commonly bilateral (45%). Unilateral cases more often affect the right hand. Hand dominance is not a factor.
- It starts with pitting and thickening of the palmar skin and underlying subcutaneous tissue, with loss of mobility of the overlying skin.
- Next a nodule forms which is firm and painless and fixed to the skin and deeper fascia. The nodule is palpable and later becomes visible.
- A cord (a linear thickening that can resemble a tendon) then develops which begins to contract over months to years.
- The contraction of the cord pulls on the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints and leads to a progressive flexion deformity in the fingers.
- Deformity can affect activities of daily living and occupation. The stage at which the patient presents will depend upon tolerance of this and is likely to depend upon the degree to which work or hobbies may be affected.
- A more aggressive form can occur in which there is early age of onset, a strong family history, bilateral hand involvement and associated ectopic disease (see below).
- Other areas of the body may be affected - eg, the knuckles (thickened knuckle pads over the dorsal surface of the PIP joints), soles of the feet (plantar fibromatosis) and the penis (penile fibromatosis, or Peyronie's disease).
- Look for:
- Early skin changes: skin thickening, dimpling, pitting
- Firm nodules (the most common site is on the distal palmar crease in line with the ring finger).
- Cords - longitudinal thickenings.
- Contractures causing flexion deformity.
- MCP joint contracture should be measured whilst applying passive extension to the PIP joint.
- PIP joint contracture is measured while the MCP joint is held in flexion.
- When there is 30° of flexion deformity at the MCP joint, the patient is unable to place their palm and fingers flat against a hard surface - for example, a table. This is known as Hueston's tabletop test.
- If there are knuckle pads on the dorsal PIP surfaces, there is more aggressive disease.
- Swan-neck deformities and boutonnière deformities may (rarely) occur.
- Trigger finger.
- Epithelioid sarcoma (rare).
- Giant cell tumour of the tendon sheath.
- Ulnar nerve palsy.
- Usually a clinical diagnosis.
- Due to the association with excess alcohol intake it may be judicious to take a drinking history and check LFTs.
- If there is any suggestion of undiagnosed diabetes then fasting blood glucose/HbA1c should be checked.
Many patients with Dupuytren's contracture do not develop significant disability and will not need treatment. For a person with contracture (who is unable to place their hand flat on a table top) or whose hand function is significantly compromised, refer them to the local hand surgery service, or to a specialist in plastic surgery or orthopaedic surgery. If there is no contracture or loss of function, there is no need to refer. Reassure the person that any pain associated with the nodules should improve over time; advise that they should return if contracture develops; if it does, referral will be indicated.
Treatment is usually considered when (or ideally before) functional disability occurs. It aims to restore hand function and prevent progression. Once contracture develops, early referral is recommended. This is because contracture and disability can become irreversible due to ligament remodelling; early referral means surgeons are then best placed to decide on the timing of any interventions. Surgical treatment is usually considered when the MCP joint is bent forwards by 30-45° and cannot be straightened; or the proximal interphalangeal joint is bent permanently by 10-20°.
Although surgical procedures such as fasciectomy or fasciotomy are often used for advanced disease, recent advances in the management of Dupuytren's disease have shown benefit of less invasive treatments, such as injectable collagenase Clostridium histolyticum (Xiapex®) and radiotherapy, particularly for early disease[6, 12]. Efficacy of injectable collagenase in the early stages of Dupuytren's contracture appears to be equivalent to surgery; however, recurrence rates are still an issue, reported as 47% at five years.
Dr Hayley Willacy draws your attention to the NICE Technology appraisal dealing with collagenase injection. People who meet the inclusion criteria for the ongoing clinical trial comparing collagenase clostridium histolyticum (CCH) with limited fasciectomy, are encouraged to participate in the study. CCH is recommended as an option for people not taking part in the trial only if all of the following apply:
- There is evidence of moderate disease.
- Percutaneous needle fasciotomy (PNF) is not considered appropriate, but limited fasciectomy is considered appropriate by the treating hand surgeon.
- The choice of treatment (CCH or limited fasciectomy) is made on an individual basis after discussion between the responsible hand surgeon and the patient about the risks and benefits of the treatments available.
- One injection is given per treatment session by a hand surgeon in an outpatient setting.
A review by the Royal College of Radiologists (RCR) states radiotherapy is effective before contracture develops and in early stages but should not be used in more advanced disease. The current National Institute for Health and Care Excellence (NICE) guidance states that the evidence on the safety and efficacy of radiation therapy for early Dupuytren's disease is limited in quantity and there is uncertainty about the natural history of early Dupuytren's disease, and therefore that the procedure should only be used with special arrangements for clinical governance, consent and audit or research.
Splinting or stretching, to prevent progression of the disease, and corticosteroid injections are not recommended.
There is not yet evidence that one treatment is superior to others.
- The procedure can be performed in an outpatient setting, using local anaesthesia.
- With closed fasciotomy, there may be short-term benefit but by three to five years the recurrence rate can be around 50%.
- It has been reviewed and approved by NICE which considers that it may be of benefit for older patients unsuitable for more major surgery.
- Nerve injury, tendon injury and infection are the main complications of the procedure, with a complication rate of 1% or less.
- This can be a segmental fasciectomy (short segments of the cord are removed), a regional fasciectomy (the entire cord is removed) or a dermofasciectomy (the cord and overlying skin are removed followed by skin grafting). Regional fasciectomy is the most common procedure undertaken for Dupuytren's contracture.
- The procedure is usually done under regional block or general anaesthetic as a day case.
- Splinting and hand physiotherapy are needed after surgery. A night splint is normally worn for three months. There is possibly evidence that this practice may adversely affect outcome - further trials are needed.
This is rarely done. It is used in severe cases (usually because presentation has been much delayed).
Dupuytren's contracture can be disabling and have a detrimental effect on work and home life.
During surgery there is a risk of nerve, tendon or blood vessel injury. Possible postoperative complications include:
- Haematoma and flap necrosis.
- Skin slough and scar contraction.
- Carpal tunnel syndrome.
- Reflex sympathetic dystrophy.
Complications of surgery have been quoted to be as high as 26%.
There are concerns about potential long-term complications of radiotherapy, such as skin cancer and sarcoma, although as yet no cases have been documented.
- The course of the disease is variable in terms of how fast and how far it will progress.
- It does not inevitably progress to contracture.
- Most patients do not require surgery.
- Surgery, when performed, improves function but postoperative recurrence may be up to 66%.
Further reading and references
; Wheeless' Textbook of Orthopaedics
; NICE Interventional Procedure Guidance, December 2016
; NICE Technology appraisal (July 2017)
; Dupuytren's disease: diagnosis and treatment. Am Fam Physician. 2007 Jul 176(1):86-9.
; NICE CKS, November 2015 (UK access only)
; Dupuytren's contracture: a retrospective database analysis to assess clinical management and costs in England. BMC Musculoskelet Disord. 2011 Apr 1212:73. doi: 10.1186/1471-2474-12-73.
; Dupuytren's contracture unfolded. BMJ. 2006 Feb 18332(7538):397-400.
; Dupuytren's Disease: Review of the Current Literature. Open Orthop J. 20115 Suppl 2:283-8. doi: 10.2174/1874325001105010283. Epub 2011 Jul 28.
; Dupuytren disease: an evolving understanding of an age-old disease. J Am Acad Orthop Surg. 2011 Dec19(12):746-57.
; Further evidence of the involvement of the Wnt signaling pathway in Dupuytren's disease. J Cell Commun Signal. 2016 Mar10(1):33-40. doi: 10.1007/s12079-015-0312-8. Epub 2015 Dec 3.
; Association among work exposure, alcohol intake, smoking and Dupuytren's disease in a large cohort study (GAZEL). BMJ Open. 2014 Jan 294(1):e004214. doi: 10.1136/bmjopen-2013-004214.
; Report by the Industrial Injuries Advisory Council (IIAC) in accordance with Section 171 of the Social Security Administration Act 1992 considering prescription for Dupuytren’s contracture in workers exposed to hand-transmitted vibration, GOV.UK, May 2014
; Heavy manual work, exposure to vibration and Dupuytren's disease? Results of a surveillance program for musculoskeletal disorders. Occup Environ Med. 2012 Apr69(4):296-9. doi: 10.1136/oemed-2011-100319. Epub 2012 Jan 2.
; Wheeless' Textbook of Orthopaedics
; Collagenase clostridium histolyticum for the treatment of Dupuytren's contracture: systematic review and economic evaluation. Health Technol Assess. 2015 Oct19(90):1-202. doi: 10.3310/hta19900.
; Dupuytren Contracture Recurrence Following Treatment With Collagenase Clostridium histolyticum (CORDLESS [Collagenase Option for Reduction of Dupuytren Long-Term Evaluation of Safety Study]): 5-Year Data. J Hand Surg Am. 2015 Aug40(8):1597-605. doi: 10.1016/j.jhsa.2015.04.036. Epub 2015 Jun 18.
; NICE Technology appraisal (July 2017)
; Royal College of Radiologists Faculty of Oncology (Dec 2015)
; NICE Interventional Procedure Guidance, December 2016
; Surgery for Dupuytren's contracture of the fingers. Cochrane Database Syst Rev. 2015 Dec 9(12):CD010143. doi: 10.1002/14651858.CD010143.pub2.
; Needle aponeurotomy for the treatment of Dupuytren's disease. Hand Clin. 2014 Feb30(1):33-8. doi: 10.1016/j.hcl.2013.09.005.
; NICE Interventional Procedure Guidance, February 2004
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