What is the pathogenesis of Palmar fibromatosis (Dupuytren contracture)?

Updated: Oct 07, 2019
  • Author: Guy J Petruzzelli, MD, PhD, MBA, FACS; Chief Editor: Gregory Gary Caputy, MD, PhD, FICS  more...
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Answer

Answer

Pathogenesis

PF is a polyclonal process and thus, a type of non-neoplastic fibroblastic proliferation. Although PF seems to have a dominant inheritance pattern, the molecular etiology of the lesion remains elusive. [59, 60]  Smoking, alcoholism, and diabetes mellitus have been associated with PF, but no definitive causal link has been made.

PF is a disease that involves two distinct fibrotic elements, a nodule and a cord. The actual pathogenesis of Dupuytren contracture remains unclear. The nodules begin as outgrowths from the fascia, and, based on electron microscopy findings, may contain myofibroblasts. As the lesions mature, the nodules become more collagenous and fibrous, with less cellular content. The resulting cord develops in the fascia, producing the familiar contracture and flexion of the fingers. Moyer and colleagues have reproduced this change from the nodule to the cord in vitro, demonstrating that serial passage of nodule fibroblasts eventually develops into cord fibroblasts. [61]  

In rare instances, these types of nodules may develop in the plantar fascia. In plantar fibromatosis, the nodules often grow more rapidly and larger. [62]  However, these nodules have less collagen deposition compared with palmar fibromatosis, and the contracture of the toes is less severe.

Because this disease has a dominant inheritance pattern, many investigators have attempted to identify molecular lesions that predispose patients to PF. The genes involved in the transforming growth factor (TGF) pathways are known to mediate proliferation in fibroblasts and are obvious molecular candidates that could be involved. However, studies have demonstrated no association between TGF genes and Dupuytren contracture. [60, 59]

Children can also present with palmar-plantar fibromatosis. In some such cases reported by Fetsch, the patient had a history of trauma, occasionally involving a foreign body. [63]

Palmar fasciitis and polyarthritis syndrome (PFPAS) is an uncommon paraneoplastic syndrome that is associated with several malignant neoplasms. In particular, it is associated with ovarian carcinoma.


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