Alerts and Notices
SynopsisKikuchi-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
Differential Diagnosis & Pitfalls
- Malignant lymphoma
- Tuberculosis, cutaneous tuberculosis
- Discoid lupus erythematosus
- Reactive lymphadenopathy due to viral infection (eg, infectious mononucleosis)
- Adult-onset Still disease / juvenile idiopathic arthritis
- Cat-scratch disease