Abstract: Objective: Cutaneous Rosai-Dorfman disease (CRDD) is a rare histiocytosis. The purpose of this study was to characterize CRDD through a retrospective clinicopathological analysis of the literature and present a new case. Methods: A literature search was performed on PubMed and information analysis was performed, which included demographics, clinical characteristics, histology, immunohistochemistry and treatment. Results: There were 183 studies available for analysis that included 371 cases. The male: female ratio was 1∶1.1, and the average age was 46 years. The disease is more common in Eastern-Asians (52.3%). The most commonly affected skin regions were limbs(43.1%) trunk(36.9%) and face (27.2%) and most lesions were multiple nodules, plaques and papules. The diagnosis of the disease mainly depends on histopathology and immunohistochemistry. The disease shows an indolent and benign course. Conclusion: Cutaneous Rosai-Dorfman disease is more common in East Asian population and it has certain clinical and pathological features. Dermatologists should improve the understanding of the disease.

Key words: Cutaneous Rosai-Dorfman disease, clinicopathological analysis