Rosai-Dorfman disease
Localization
lymph node
skin (12558621), hypodermis (1498408, 3401030)
pleura (14652824)
intracranial (14608579, 12637307), brain (12601463), meninges
nasal region (12761707)
breast (12760958, 9416494, 9199644)
thyroid (12699598, 12683901)
bone (12652340, 9653919, 9526780, 2013464)
gastrointestinal tract (12562257 10746683, 17400399)
eye (12479625), lacrymal gland
kidneys (10587134, 10471758, 9072605, 1456886)
pancreas (10370692, 11555773)
salivary glands (9472588)
liver (8771474)
larynx
mostly isolated
rarely multicentric (12622452)
Synopsis
large, pale, histiocytic cells (Rosai-Dorfman cells) exhibiting emperipolesis (cytophagocytosis)
Immunohistochemistry
S-100+
CD68+
CD1a-
Electron microscopy
Birbeck granules are absent
Associations
acute lymphoblastic leukemia (11496358#)
lymphoma (9031615, 9006752)
follicular lymphoma (10786808)
nodular lymphocyte predominant Hodgkin disease (10786808, 8522313)
chronic autoimmune thyroiditis (10710276)
inflammatory pseudotumor (9059366)
systemic lupus erythematosus (11555773)
References