A rare case of purely cutaneous Rosai–Dorfman’s disease: Xanthoma-like presentation

Rosai–Dorfman’s disease (RDD) is sinus histiocytosis with massive lymphadenopathy. Cutaneous RDD is a rare entity that presents with isolated skin involvement without any systemic involvement. We report a 35-year-old man with an asymptomatic large nodule on the preauricular region of the face with an irregular surface and multiple yellow studded nodules. On detailed physical and systemic examination, there was no lymph node or systemic involvement. Skin biopsy on histopathology showed dense dermal inflammatory infiltrate and foamy histiocytes with emperipolesis. Immunohistochemical markers were positive for S100 protein and CD68 protein but negative for CD1a. Thus, a diagnosis of cutaneous RDD was made.