Abstract: Mao Qiuxia, Zhang Wanlu, Li Wenrui, Zhao Sijia, Sun Jianfang, Cui Pan′gen, Li Chengrang Department of Dermatology, Hospital of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China （Mao QX ［current affiliation: Department of Dermatology, Jiangyin Hospital of Traditional Chinese Medicine, Affiliated to Nanjing University of Chinese Medicine, Jiangyin 214400, Jiangsu, China］, Zhang WL, Li WR, Zhao SJ, Cui PG, Li CR）; Department of Pathology, Hospital of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China （Sun JF） Corresponding author: Li Chengrang, Email: nylcr72@163.com 【Abstract】 Three cases of dermatomyositis presenting with inverse Gottron′s papules were reported.Of the 3 patients, there were 2 males and 1 female aged 43, 41 and 46 years respectively, whose disease durations were 1, 6, 7 months respectively. Inverse Gottron′s papules manifested as papules overlying the palmar aspect of the metacarpal and interphalangeal joints, which were arranged in a linear pattern in 2 cases. Of the 3 cases, 1 presented with interstitial pneumonia, 3 with myositis, and 2 showed negative anti-nuclear antibody test. Histopathological examination of inverse Gottron′s papules in 1 case revealed focal liquefaction degeneration of basal cells and perivascular infiltration with inflammatory cells, especially lymphocytes, in the superficial dermis. The 3 patients received oral glucocorticoids and immunosuppressive agents in the early period of treatment. After 3-month treatment, clinical symptoms including muscle weakness and muscle pain were improved evidently, but no obvious improvement in inverse Gottron′s papules was observed. After 1-year treatment, these papules on the palmar aspect were markedly relieved in 1 case.