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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