Abstract: Rosai-Dorfman disease (RDD) can involve skin, parotid gland, eyelid, liver or breast, but the disease involving skin, eyes and bone at the same time has not been reported. We reported a case of RDD involving skin, eyes and bone at the same time. The patient was a 41-year-old male presented with numbness on the right face for 3 months, followed by purplish-red papules on the same area for 1 month, without systemic involvement at the moment. Histological examination showed infiltration of lymphocytes, plasma cells, giant cells, and neutrophils in the dermis. Immunohistochemistry revealed that the infiltrated cells were positive for S100, CD68 and all cells were negative for CD1a. The diagnosis of cutaneous Rosai-Dorfman disease was made. After 2 months of treatment with oral thalidomide, topical hydrocortisone butyrate cream and tacrolimus ointment, the lesions subsided. But after 7 months, the hyperplasia involved the orbit and destroyed the bone, the patient went to ophthalmology department for surgery and the patient is under followed up.

Key words: cutaneous Rosai-Dorfman disease, orbital Rosai-Dorfman disease, bony destruction