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. CONCLUSION: Our data showed increased expression of CTGF in the sclerotic fibroblasts of nodular scleroderma, suggesting that CTGF plays an important role in its pathogenesis.