| Rosai-Dorfman disease | |
|---|---|
| Classification and external resources | |
Micrograph of a lymph node involved by Rosai-Dorfman disease showing emperipolesis. H&E stain. |
|
| ICD-10 | D76.3 (ILDS D76.360) |
| DiseasesDB | 31419 |
Rosai–Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare disorder of unknown etiology that is characterized by the overproduction of histiocytes, which accumulate in lymph nodes throughout the body.[1]:747 Lymphadenopathy of the neck is the most common place of histiocyte accumulation, although accumulation outside of lymph nodes may occur, as well. The most common sites of accumulation outside of the lymph nodes are skin, upper respiratory tract, and the sinuses.[2][3] The symptoms of this disease vary with the site of accumulation similar to other regional tumors. For instance, accumulation in closed spaces such as the cranium can lead to poor outcomes compared to growth in the dermis of an extremity where surgical excision is possible. This condition has been named after Ronald F. Dorfman and Juan Rosai. An alternative eponym of this condition is known as Destombes-Rosai-Dorfman syndrome, part of which is named after Pierre-Paul Louis Lucien Destombes