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 Table of Contents  
Year : 2018  |  Volume : 13  |  Issue : 1  |  Page : 71-72

Kikuchi-Fujimoto Disease (Histiocytic Necrotizing Lymphadenitis) associated with aseptic meningitis

1 Department of Pediatrics, Manipal Hospital, Bengaluru, Karnataka, India
2 Pediatric Rheumatology Clinic, Department of Pediatrics, Manipal Hospital and Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India

Date of Web Publication26-Feb-2018

Correspondence Address:
Dr. Anand Prahalad Rao
Pediatric Rheumatology Clinic, Manipal Hospital and Indira Gandhi Institute of Child Health, Bengaluru, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_119_17

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How to cite this article:
Deepa C M, Rao AP. Kikuchi-Fujimoto Disease (Histiocytic Necrotizing Lymphadenitis) associated with aseptic meningitis. Indian J Rheumatol 2018;13:71-2

How to cite this URL:
Deepa C M, Rao AP. Kikuchi-Fujimoto Disease (Histiocytic Necrotizing Lymphadenitis) associated with aseptic meningitis. Indian J Rheumatol [serial online] 2018 [cited 2021 Jun 18];13:71-2. Available from:

Dear Editor,

We report a 6-year-old male child who was brought to hospital with complaints of fever, headache, photophobia, recurrent vomiting, and fatigue of 4-week duration. Physical examination showed generalized tender lymphadenopathy, bilateral periorbital puffiness, conjunctival congestion, and splenomegaly. Clinical signs of meningitis were absent in the boy. Blood tests revealed normal white blood cell counts along with raised erythrocyte sedimentation rate (ESR) and normal C-reactive protein (CRP). The cerebrospinal fluid (CSF) analysis revealed pleocytosis (160 cells), all mononuclear cells along with depleted CSF glucose and raised CSF protein levels. Aseptic meningitis was considered; however, the patient was started on intravenous (IV) antibiotics. Magnetic resonance imaging of the brain was normal and CSF culture was negative. Mantoux test was anergic, and chest X-ray was normal. The child continued to be febrile and persisted to have headache. The child had been diagnosed to have  Kikuchi-Fujimoto disease More Details (KFD) couple of years before the current episode on the basis of histopathology of the lymph node revealing necrotizing lymphadenitis. In view of history of KFD and literature review revealing KFD's association with aseptic meningitis, aseptic meningitis secondary to KFD was suspected, and the child was given IV steroid pulse therapy followed by oral steroids. His antinuclear antibodies by immunofluorescence were positive at a titer of 1:320, which turned negative on follow-up. His fever and meningismus improved rapidly, and the steroids were stopped in 3 months' time after gradual taper. He continues to be on a close follow-up since the past 4 years and has had two further flares (febrile illness with tender lymphadenopathy with transaminitis), but no further episodes of aseptic meningitis have been noted.

Kikuchi disease (KFD or histiocytic necrotizing lymphadenitis) is a benign condition characterized by febrile illness and cervical lymphadenopathy. Although the etiological basis of the disease is unknown, it has been known to be associated with a few connective tissue disorders, especially systemic lupus erythematosus (SLE). SLE has been known to precede, happen concurrently as well as postcede the KFD.[1] Cervical lymphadenopathy affects 56%–98% of patients with KFD. Nearly 2.8%–9.8% of KFD cases present with associated aseptic meningitis.[2] Meningitis should be considered correlating and not coincidental to KFD. A case series of aseptic meningitis from Japan involving 11 patients revealed that majority of the patients have had a diagnosis of KFD before they had aseptic meningitis (9 out of 11)[2] similar to our case. Mild-to-severe headache has been described in most of the patients. The absence of meningeal signs in 45% of these patients may lead to significant under diagnosis. Leukopenia, elevated ESR, and positive CRP were seen in most of the patients in the series by Sato et al. Brain imaging was nearly normal in all patients. CSF analysis in most of the patients revealed mild-to-moderate pleocytosis (61–1685 cells/cu mm) with elevated protein and decreased glucose values.[2] Serological tests as well as CSF cultures for viral, mycobacterial, and fungal infections were reported to be negative.[2] Mantoux test was negative in all these patients. Natural resolution in a matter of 1–4 months is the norm even without any treatment. Patients normally respond to IV steroids within 24–48 h.[1],[2],[3],[4] Neurological sequelae are uncommon. Tuberculous meningitis[5] and aseptic meningitis associated with rickettsial fever would be differential diagnoses of this condition. Diagnosis of KFD is more presumptive, and basis for making the diagnosis is previous history of KFD in the child or the finding of necrotizing lymphadenitis on biopsy in a child with meningitis.

In children with aseptic meningitis-like picture with tender lymphadenopathy with or without transaminitis, KFD should be suspected. Treatment using corticosteroids in such patients would help in quick recovery.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M, et al. Kikuchi-Fujimoto disease: Analysis of 244 cases. Clin Rheumatol 2007;26:50-4.  Back to cited text no. 1
Sato Y, Kuno H, Oizumi K. Histiocytic necrotizing lymphadenitis (Kikuchi's disease) with aseptic meningitis. J Neurol Sci 1999;163:187-91.  Back to cited text no. 2
Gonçalves LF, Debelenko LV, Bhambhani KJ, Scheid A, Altinok D. Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease) with CNS involvement in a child. Pediatr Radiol 2014;44:234-8.  Back to cited text no. 3
Khishfe BF, Krass LM, Nordquist EK. Kikuchi disease presenting with aseptic meningitis. Am J Emerg Med 2014;32:1298.e1-2.  Back to cited text no. 4
Noursadeghi M, Aqel N, Pasvol G. Kikuchi's disease: A rare cause of meningitis? Clin Infect Dis 2005;41:e80-2.  Back to cited text no. 5

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2 Recurrent Aseptic Meningitis Associated with Kikuchięs Disease (Histiocytic Necrotizing Lymphadenitis): A Case Report and Literature Review
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