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Lu Jiaying, Wu Wenyu. Department of Dermatology, Huashan Hospital, Fudan University, Shanghai 200040, China Corresponding author: Wu Wenyu, Email: wuwenyu@medmail.com.cn ��Abstract�� Localized scleroderma is a chronic skin disease characterized by local fibrosis of skin and subcutaneous tissue. The etiology of localized scleroderma is unknown. Damage to small blood vessels, activation of T lymphocytes and overproduction of connective tissue may contribute to the pathogenesis of localized scleroderma. The activation of T lymphocytes can lead to the release of several kinds of cytokines and chemokines, including tumor necrosis factor-α, transforming growth factor-β, soluble interleukin-2 receptor, soluble interleukin-6 receptor, etc. Damage to vascular endothelial cells can also mediate the release of cytokines associated with fibrosis. Both differences and links exist between the pathogenesis of localized scleroderma and that of systemic scleroderma. The authors summarize latest progress in the pathogenesis of localized scleroderma.