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Hu Bin, Zhou Lu, Chen Hao, Wen Sijian, Wu Kan, Sun Jianfang. Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College; Jiangsu Key Laboratory of Molecular Biology for Skin Diseases and STIs, Nanjing 210042, China Corresponding author: Sun Jianfang, Email: sunjf57@163.com ��Abstract�� Objective To further investigate the clinical and histopathological characteristics of stiff skin syndrome ��SSS��. Methods The clinical manifestation, histopathological characteristics and treatment response of SSS were analyzed retrospectively in five patients. Results The age of clinic visits varied from 5 to 9 years, the age at onset of SSS from 3 months to 4 years, and clinical course from 2 to 6 years in these patients. SSS was characterized by stone-hard indurations of skin of unilateral lower limbs, the abdomen and buttocks, as well as mild hypertrichosis and limitation of joint mobility. Histopathological examination revealed collagen fiber hyperplasia in the dermis with mucopolysaccharide deposition. Medical treatment resulted in no obvious improvement. Conclusions SSS often occurs at birth or in early infancy with the involvement of unilateral limbs in most cases and bilateral limbs in some cases. It should be differentiated from some diseases such as scleroderma. No effective treatment is available for SSS at present.