Rosai-Dorfman disease

  • Macrophage (vs dendritic cell) disorder
  • “Sinus histiocytosis with massive lymphadenopathy”
  • Children and young adults
  • Skin involvement in <10%, nonspecific, polymorphic
  • Massive, painless, bilateral cervical lymphadenopathy (not in all cases)
  • Fever, neutrophilia, anemia, ESR, polyclonal hypergammaglobulinemia (EBV/HHV6 reactive?)
  • Pathology
    • Dermal large foamy and epithelioid histiocytes emperipolesis (engolfement of cells inside other cells, not necessarily destroying)
    • CD68 and S100+
    • Sclerosis
    • Plasma cells
  • Indolent course, spontaneous regression over years, prednisone helpful for acute symptoms