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Du Wei, Sun Qiuning, Xing Zeyu, Fang Kai, Qu Tao. Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China Corresponding author: Qu Tao, Email: qutao_pumch@163.com ��Abstract�� Objective To assess the clinicopathological characteristics of stiff skin syndrome. Methods The clinical and histopathological manifestations of 21 patients with stiff skin syndrome were retrospectively studied. Results There were 7 males and 14 females among the 21 patients. The average age at onset was 5.2 ± 4.2 years. Buttocks, thighs, waist, shoulders and back were the most frequently involved sites in these patients. Clinical manifestations mainly included skin stiffness, localized hypertrichosis and pigmentation. Limited joint mobility was also observed in some patients. Histological examination showed an increase in the number and diameter of collagen fibers in the dermis without infiltration of inflammatory cells. Conclusions Stiff skin syndrome often occurs at birth or in early childhood with skin stiffness, limited joint mobility, and hypertrichosis as the most common clinical manifestations.