Epidemiology and pathogenesis of scleroderma

• Published in: Australasian journal of dermatology Vol. 44; no. 1; pp. 1 - 7
• Main Authors: Chen, Keng, See, Adrian, Shumack, Stephen
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Science Pty 01-02-2003; Blackwell
• Subjects: Biological and medical sciences; connective tissue; environment; fibrosis; genetics; Humans; immune system; localized scleroderma; Medical sciences; microchimerism; morphoea; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Scleroderma, Localized - epidemiology; Scleroderma, Localized - etiology; Scleroderma, Localized - immunology; Scleroderma, Systemic - epidemiology; Scleroderma, Systemic - etiology; Scleroderma, Systemic - immunology; systemic sclerosis; Human; Connective tissue disease; Skin disease; Pathogenesis; Circumscribed scleroderma; Review; Epidemiology; Connective tissue; localized scleroderma; systemic sclerosis; morphoea; Fibrosis; Environment; Genetics; microchimerism; Immune system
• ISSN: 0004-8380; 1440-0960
• DOI: 10.1046/j.1440-0960.2003.06301.x

SUMMARY
Scleroderma is a heterogenous connective tissue disorder characterized by fibrosis of the skin, with or without internal organ involvement. The aetiology of scleroderma may involve both environmental and genetic factors. Abnormalities involving the immune system, vascular tissue and extracellular matrix have been demonstrated. Recent research has focused on microchimerism as a risk factor for the development of scleroderma. This article reviews the epidemiology and pathogenesis of this disorder.