We report a case of cutaneous Rosai-Dorfman disease (RDD) rising in a 52-year-old Japanese female with two nodules: one was a red nodule on her cheek and the other was a subcutaneous nodule on her chest. Histopathological analysis revealed that the nodules were composed of many histiocytes that were positive to S100, but negative to CD1a showing “emperipolesis”. We diagnosed her with cutaneous RDD based on the histological findings since she had no other lymphadenopathy or extranodal involvement. RDD derived from the skin without lymph node involvement is rare worldwide with approximately 50 cases reported. No cases have been reported in Japan. [Skin Cancer (Japan) 2004; 19: 124-127]
