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 Table of Contents  
IMAGES IN ACADEMIC MEDICINE
Year : 2019  |  Volume : 5  |  Issue : 3  |  Page : 191-193

Dupuytren's contracture: Concise approach to an enigmatic disease


1 Department of Internal Medicine, Lewis Katz School of Medicine at Temple University, Philadelphia, USA
2 Department of Cardiac Electrophysiology, St. Luke's University Health Network, Bethlehem, Pennsylvania, USA

Date of Submission12-Mar-2019
Date of Decision28-Mar-2019
Date of Acceptance30-Apr-2019
Date of Web Publication24-Dec-2019

Correspondence Address:
Dr. Sudip Nanda
Department of Cardiac Electrophysiology, St. Luke's University Health Network, 801 Ostrum Street, Bethlehem, Pennsylvania 18015
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJAM.IJAM_12_19

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  Abstract 


Dupuytren's disease (DD) is a debilitating fibroproliferative disorder of the palmar fascia. The clinical approach and management of DD is often challenging for clinicians. The purpose of this image in academic medicine is to review the pathophysiology, diagnosis, and treatment of DD.
The following core competencies are addressed in this article: Patient care, Medical knowledge.

Keywords: Approach, contracture, diagnosis, disease, Dupuytren, enigmatic, treatment


How to cite this article:
Noto JG, Johnson JA, Nanda S. Dupuytren's contracture: Concise approach to an enigmatic disease. Int J Acad Med 2019;5:191-3

How to cite this URL:
Noto JG, Johnson JA, Nanda S. Dupuytren's contracture: Concise approach to an enigmatic disease. Int J Acad Med [serial online] 2019 [cited 2020 Nov 27];5:191-3. Available from: https://www.ijam-web.org/text.asp?2019/5/3/191/273928




  Introduction Top


Dupuytren's disease (DD) is a debilitating fibroproliferative disorder of the palmar fascia.[1] It presents with palpable nodules and fibrous cords, which progress to permanent flexion contracture of the affected digits.[2] The incidence of DD varies considerably depending on the population studied.[3] It is highest in Caucasian males and increases with advancing age.[3] The management of DD is often challenging.[4] It is therefore essential for clinicians to be familiar with the clinical approach to DD. The purpose of this image in academic medicine is to present a concise approach to the pathophysiology, diagnosis, and treatment of DD.


  Case Report Top


A 63-year-old right-hand-dominant male with a medical history of hyperlipidemia and atrial flutter presents with progressive bilateral loss of extension in several fingers. He had no family history of similar conditions. On physical examination, the patient was asked to fully extend his fingers, which revealed the finding in [Figure 1]. There was no evidence of ectopic fibrotic disease on examination. How would you approach this patient's concerns?
Figure 1: Severe bilateral Dupuytren's disease

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  Discussion Top


The exact etiology of DD is unknown. It is thought to arise from a combination of genetic and environmental factors.[1] Luck characterized the three histological stages of DD: proliferative, involutional, and residual.[2] The proliferative stage is characterized by a focus of fibroblast proliferation between the superficial palmar fascia and the skin. This results in the formation of a palpable nodule. In the involutional stage, the nodule begins to shrink, becoming less cellular and more collagenous. It places tension on the overlying skin, which begins to draw the affected joint into flexion. In the residual stage, the nodule disappears, leaving a fibrous cord anchoring the skin to the underlying fascia.[1],[2]

The earliest clinical manifestation of DD is the painless palmar nodule. Over the course of several months to years, extension of the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints progressively becomes limited. Nodules are eventually replaced by cords, which permanently anchor the affected digit in a flexion contracture [Figure 1].[4] The fourth and fifth digits are usually affected, and bilateral involvement is common.[2] Patients will often present with difficulty with handshake, manual labor, or activities of daily living.[4] In some cases, ectopic fibrotic disease may be present on the dorsal hand (Garrod's knuckle pads), plantar foot (Ledderhose's disease), or dorsal penis (Peyronie's disease).[5]

The differential diagnosis of suspected DD is shown in [Table 1]. It is usually distinguished from similar diseases by the presence of painless nodules, irreducible contracture, and predilection for the fourth and fifth digits. The history should identify the rate of disease progression, the degree of disability imposed by it, and family history of DD. The physical examination should determine the severity of the disease. If flexion contractures are present, the MCP and PIP joint angles should be measured with a goniometer. The clinician should look for the presence of ectopic disease. A positive Hueston's tabletop test (in which the patient is unable to place their palms flat on a surface) is a good indication for referral to a hand specialist.[1]
Table 1: Differential diagnosis in patients with suspected Dupuytren's disease

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Approximately 10% of cases of DD will spontaneously regress without intervention.[9] For patients with early-stage disease, Clostridium histolyticum collagenase injections have been shown to improve contractures.[10] Surgery may be indicated in patients with functionally symptomatic disease or for >40° flexion contracture at the MCP or 20° at the PIP joint.[1],[11] For early-stage disease, percutaneous needle aponeurotomy, an in-office procedure in which the surgeon divides the cord with a needle under local anesthesia, may be performed. For moderately severe cases, open fasciectomy may be performed, in which the surgeon excises the cord and the nodule. For severe refractory cases, dermatofasciectomy involves removing the cord and overlying skin with subsequent skin graft placement.[1] With all interventions for DD, recurrence is common.[1]

New research has implicated tumor necrosis factor-alpha (TNF-alpha) in the pathogenesis of DD.[12] TNF-alpha induces the differentiation of fibroblasts into contractile myofibroblasts in genetically susceptible patients.[12] As such, the anti-TNF antibody adalimumab has been suggested as a therapeutic option in early-stage disease to prevent progression.[12] A recent phase 2a clinical trial demonstrated downregulation of the myofibroblast phenotype in the palmar dermis of patients injected with adalimumab when compared to placebo.[13] As the safety profile of adalimumab is well established, it represents a promising new therapeutic option for patients with DD.[12],[13]


  Conclusion Top


We opened with the case of a 63-year-old male presenting with advanced stage DD. As this patient has a positive Hueston's tabletop test and significant functional impairment, he should be referred to a hand specialist. In all cases of DD, a thorough history and physical examination should be performed to rule out similar diseases. The mainstay of treatment in DD is surgery, but less invasive options are emerging.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Ethical conduct of research

This research did not require Ethics Committee approval because it did not involve human or animal experimental designs. Applicable EQUATOR Network (http://www.equator-network.org) research reporting guidelines were followed.



 
  References Top

1.
Shih B, Bayat A. Scientific understanding and clinical management of Dupuytren disease. Nat Rev Rheumatol 2010;6:715-26.  Back to cited text no. 1
    
2.
Luck JV. Dupuytren's contracture; a new concept of the pathogenesis correlated with surgical management. J Bone Joint Surg Am 1959;41-A:635-64.  Back to cited text no. 2
    
3.
Hindocha S, McGrouther DA, Bayat A. Epidemiological evaluation of Dupuytren's disease incidence and prevalence rates in relation to etiology. Hand (N Y) 2009;4:256-69.  Back to cited text no. 3
    
4.
Townley WA, Baker R, Sheppard N, Grobbelaar AO. Dupuytren's contracture unfolded. BMJ 2006;332:397-400.  Back to cited text no. 4
    
5.
Hindocha S, Stanley JK, Watson JS, Bayat A. Revised Tubiana's staging system for assessment of disease severity in Dupuytren's disease-preliminary clinical findings. Hand (N Y) 2008;3:80-6.  Back to cited text no. 5
    
6.
Singh V, Haq A, Priyadarshini P, Kumar P. Camptodactyly: An unsolved area of plastic surgery. Arch Plast Surg 2018;45:363-6.  Back to cited text no. 6
    
7.
Cherqaoui R, McKenzie S, Nunlee-Bland G. Diabetic cheiroarthropathy: A case report and review of the literature. Case Rep Endocrinol 2013;2013:257028.  Back to cited text no. 7
    
8.
Makkouk AH, Oetgen ME, Swigart CR, Dodds SD. Trigger finger: Etiology, evaluation, and treatment. Curr Rev Musculoskelet Med 2008;1:92-6.  Back to cited text no. 8
    
9.
Gudmundsson KG, Arngrimsson R, Jónsson T. Eighteen years follow-up study of the clinical manifestations and progression of Dupuytren's disease. Scand J Rheumatol 2001;30:31-4.  Back to cited text no. 9
    
10.
Hurst LC, Badalamente MA, Hentz VR, Hotchkiss RN, Kaplan FT, Meals RA, et al. Injectable collagenase clostridium histolyticum for Dupuytren's contracture. N Engl J Med 2009;361:968-79.  Back to cited text no. 10
    
11.
Au-Yong IT, Wildin CJ, Dias JJ, Page RE. A review of common practice in Dupuytren surgery. Tech Hand Up Extrem Surg 2005;9:178-87.  Back to cited text no. 11
    
12.
Verjee LS, Verhoekx JS, Chan JK, Krausgruber T, Nicolaidou V, Izadi D, et al. Unraveling the signaling pathways promoting fibrosis in Dupuytren's disease reveals TNF as a therapeutic target. Proc Natl Acad Sci U S A 2013;110:E928-37.  Back to cited text no. 12
    
13.
Nanchahal J, Ball C, Davidson D, Williams L, Sones W, McCann FE, et al. Anti-tumour necrosis factor therapy for Dupuytren's disease: A randomised dose response proof of concept phase 2a clinical trial. EBioMedicine 2018;33:282-8.  Back to cited text no. 13
    


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