Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto Disease, KFD) is a disorder of unknown etiology. It was initially described in Asia but has been seen worldwide. Its occurrence may be underestimated.


Clinical features:

(1) fever of unknown origin (FUO)

(2) systemic symptoms such as malaise and weight loss

(3) lymphadenopathy, usually cervical but occasionally elsewhere

(4) variable anemia with elevated ESR

(5) self-limited course with spontaneous resolution in 2 to 4 months


Pathologic features:

(1) necrotizing lymphadenitis with histiocytes, plasma cells and CD8-positive T cells

(2) absence of neutrophils


Diagnosis requires exclusion of other conditions including:

(1) malignant lymphoma

(2) systemic lupus erythematosus (SLE)


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