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Dupuytren's contracture

Authors
Rohit Aggarwal, MD, MSc
Philip E Blazar, MD
Section Editor
Zacharia Isaac, MD
Deputy Editor
Monica Ramirez Curtis, MD, MPH

INTRODUCTION

Dupuytren’s contracture is a relatively common disorder characterized by progressive fibrosis of the palmar fascia [1]. It is a benign, slowly progressive fibroproliferative disease of the palmar fascia. Initial fascial thickening is usually seen as a nodule in the palm, which can be painful or painless and often goes unnoticed and undiagnosed. Joint stiffness and a loss of full extension develop insidiously over decades.

As the process evolves, nodules progress to form longitudinal bands referred to as cords on the palmar fascia, and the finger gradually loses extension, with contractures that draw one or more fingers into flexion at the metacarpophalangeal (MCP) joint (picture 1), proximal interphalangeal (PIP) joint, or both [2]. The term Dupuytren disease (DD) is also used for this disorder, as the fingers are not always held in a fixed flexion deformity.

ETIOLOGY

The cause of Dupuytren’s contracture is unknown; important factors include genetics, ethnicity, sex, and age and may include certain environmental factors and other diseases [3]. The disorder, which most affects those of northern European ancestry, appears to have a pronounced genetic predisposition; 68 percent of male relatives of affected patients develop the disease. In a study involving patients from the Netherlands, Germany, and the United Kingdom, six of nine genetic loci found associated with genetic susceptibility to Dupuytren’s disease contained genes encoding proteins in the Wnt-signaling pathway [4]. Overstimulation of this pathway, which can regulate cellular proliferation, could potentially lead to fibroblast proliferation and nodule formation in this disorder through effects upon beta-catenin [5].

Pathologically, Dupuytren’s contracture is characterized by fibroblastic proliferation and disorderly collagen deposition with fascial thickening. Formation of a nodule or nodules occurs in the early proliferative stage of the disease and is the pathognomonic lesion of Dupuytren’s contracture. Nodules form due to proliferation of fibroblasts in the superficial palmar fascia and histologically are composed of fibroblasts and type III collagen. Smooth muscle fibroblasts and myofibroblasts are present in the nodules; increased concentrations of prostaglandins are also found within the nodules and may influence myofibroblast contractility [6]. The flexor tendons are not intrinsically involved, but invasion of the dermis occurs and results in characteristic puckering and tethering of the skin.

The presence of CD3-positive lymphocytes and the expression of major histocompatibility complex (MHC) class II proteins also suggest a possible role for a T-cell mediated autoimmune response in this disorder [7].

              

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Literature review current through: Nov 2016. | This topic last updated: Tue Aug 04 00:00:00 GMT+00:00 2015.
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