Rosai–Dorfman disease

Rosai-Dorfman disease
Other namesDestombes-Rosaï-Dorfman disease
Patient with enlarged lymph node on neck caused by Rosai-Dorfman disease; H&E stain of fine-needle aspiration specimens showed histiocytes with intact lymphocytes and plasma cells inside them (i.e., emperipolesis).
SpecialtyHematology 

Rosai–Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy or sometimes as Destombes–Rosai–Dorfman disease, is a rare disorder of unknown cause that is characterized by abundant histiocytes in lymph nodes or other locations including the skin, sinuses, brain and heart. Individuals with the disorder often present with enlarged lymph nodes and a nodular red skin rash. The main causes of morbidity with the illness are systemic infection from impaired immune response and organ dysfunction from histiocyte deposition throughout the body.

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