Cutaneous Rosai-Dorfman Disease - A Rare Non-Langerhans Cell Histiocytosis

Abstract

Cutaneous Rosai-Dorfman disease is classified as non-Langerhans cell histiocytosis. It is a benign lymphoproliferative disorder involving only skin and subcutaneous tissue, which is rare and not well documented. It manifests as erythematous to brown papules, plaques, or nodules with histiocyte-rich inflammatory infiltrate which constantly exhibit emperipolesis i.e., uptake of intact lymphocytes and plasma cells; they express both Langerhans cell and macrophage markers (S100 and CD68 respectively). We report a case of a 45-year-old female presenting with a plaque on right cheek since 10 months without systemic symptoms. The lesion was excised in-toto and defect covered with rhomboid flap repair.

Keywords

• Histiocytosis
• Emperipolesis
• Cutaneous Rosai-Dorfman