title: Nodular scleroderma: increased expression of connective tissue growth factor.
abstract: BACKGROUND
  Nodular scleroderma is a fibrosing reaction, presenting well-defined, firm, raised nodules, which resembles keloid. Recent studies suggest that connective tissue growth factor (CTGF) is involved in the fibrotic process, and its expression is increased in many fibrosing diseases.
  
  
  OBJECTIVE
  In this study, we report 3 cases of nodular scleroderma, and the expression of CTGF is also investigated.
  
  
  METHODS
  Three patients who developed nodular scleroderma are described. Expression of CTGF protein and transcripts in nodular scleroderma was examined by immunohistochemistry and in situ hybridization.
  
  
  RESULTS
  Patients were 1 female and 2 males, and all cases were associated with diffuse systemic sclerosis. Nodular lesions were found on the chest (2 cases), back (1 case), upper arm (1 case) and nape (1 case). Histology of the nodular lesions showed thickened collagen bundles in the dermis. Results of immunohistochemistry showed that expression of CTGF was detected on the sclerotic fibroblasts in the lesions of nodular scleroderma, while it was not detected in fibroblasts in normal skin. In situ expression of CTGF transcripts was abundantly detected on the fibroblastic cells in nodular scleroderma.
  
  
  CONCLUSIONS
  Our data showed increased expression of CTGF in the sclerotic fibroblasts of nodular scleroderma, suggesting that CTGF plays an important role in its pathogenesis.
authors.affiliation: Department of Dermatology, Tokyo Medical and Dental University, School of Medicine, Japan. yamamoto.derm@med.tmd.ac.jp
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Yamamoto
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Sawada
authors.forename: Ichiro
authors.initials: I
authors.lastname: Katayama
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Nishioka
mesh_terms: D000328:Adult; D055513:Connective Tissue Growth Factor; D005260:Female; D006801:Humans; D017874:Immediate-Early Proteins / Q000096:biosynthesis*; D036341:Intercellular Signaling Peptides and Proteins / Q000096:biosynthesis*; D008297:Male; D008875:Middle Aged; D012594:Scleroderma, Localized / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D014945:Wound Healing / Q000502:physiology
chemical_list: C526317:CCN2 protein, human; D017874:Immediate-Early Proteins; D036341:Intercellular Signaling Peptides and Proteins; D055513:Connective Tissue Growth Factor