Last updated: November 4, 2014
Synonyms: Kikuchi-Fujimoto disease, histiocytic necrotizing lymphadenitis.
ICD-9 Code: 683.0.
Definition: A benign, rare disorder that affects young women (more so than men) with recurrent necrotizing lymphadenitis often associated with other systemic inflammatory or infectious disorders.
Pathology: Lymph node biopsies show histiocytic necrotizing lymphadenitis, fragmentation, necrosis, karyorrhexis zonation, and absence of neutrophils and predominance of histiocytes, T lymphocytes with many plasmacytoid monocytes. Immunohistochemistry may be necessary to distinguish Kikuchi’s from malignant lymphoma.
Risk Factors: Kikuchi’s disease may be seen in association with lupus, Still’s disease, interstitial lung disease, hemophagocytic syndrome, mononeuritis, or infections (e.g., tuberculosis, HIV, Parvovirus, Brucella).
Cardinal Findings: Affects adolescents and young adults (<40 years) with painful cervical lymphadenopathy, often associated with fever, flu-like symptoms, weight loss, and night sweats, and uncommonly demonstrates arthritis or hepatosplenomegaly.
Diagnostic Tests: Tests are normal or show nonspecific elevations of ESR, CRP, and hepatic enzymes. Lactate dehydrogenase is usually elevated and 20% to 50% will show lymphopenia.
Differential Diagnosis: Lupus, infective lymphadenopathy, and lymphoma.
Therapy: Supportive care and corticosteroids are primarily used.
Prognosis: Kikuchi’s disease is a self-limiting disease that will spontaneously remit in 4 to 6 months, although lymph nodes may persist longer.
Chen YH, Lan JL. Kikuchi disease in systemic lupus erythematosus: clinical features and literature review. J Microbiol Immunol Infect 1998;31:187–192. PMID:10496156