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Kikuchi-Fujimoto disease
Other Resources UpToDate PubMed

Kikuchi-Fujimoto disease

Contributors: Deborah Paul MD, Connie R. Shi MD, Susan Burgin MD
Other Resources UpToDate PubMed


Kikuchi-Fujimoto disease (KFD), also known as Kikuchi disease or histiocytic necrotizing lymphadenitis, is a rare condition presenting with fever, tender lymphadenopathy (typically posterior cervical), and occasionally a rash on the face, upper extremities, or trunk.

The condition is most common in young adults (under age 30) of Japanese and other Asian descent, although cases in all ethnic groups have been reported. Children can also be affected.

The cause of KFD is not known. There have been proposed associations with viral infections such as Epstein-Barr virus (EBV). Associations between KFD and systemic lupus erythematosus (SLE) – with KFD sometimes preceding diagnosis of SLE – have also been reported. Human leukocyte antigen (HLA) class II alleles (HLA-DPA1 and HLA-DPB1) in KFD patients are more frequently present than in the general population.

KFD is self-limited and typically remits spontaneously in 1-4 months in most patients.


I88.1 – Chronic lymphadenitis, except mesenteric

127217009 – Histiocytic necrotizing lymphadenitis

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Differential Diagnosis & Pitfalls

  • Malignant Lymphoma
  • Tuberculosis, Cutaneous tuberculosis
  • Systemic lupus erythematosus
  • Discoid lupus erythematosus
  • Reactive lymphadenopathy due to viral infection (eg, infectious Mononucleosis)
  • Adult onset Still disease / Juvenile idiopathic arthritis
  • Cat-scratch disease

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Last Reviewed:11/19/2020
Last Updated:12/03/2020
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Kikuchi-Fujimoto disease
A medical illustration showing key findings of Kikuchi-Fujimoto disease : Diarrhea, Fever, Nausea/vomiting, Malaise, Tender lymphadenopathy, Sore throat, Smooth papules, Blanching macules, Temporal headache
Clinical image of Kikuchi-Fujimoto disease - imageId=5618467. Click to open in gallery.  caption: 'Crusted and scaly papules and plaques in and around the ear.'
Crusted and scaly papules and plaques in and around the ear.
Copyright © 2024 VisualDx®. All rights reserved.