Case Report: Histiocytic Necrotizing Lymphadenitis (Kikuchi–Fujimoto Disease) Concurrent With Aseptic Meningitis

Abstract

Kikuchi–Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, benign, self-limiting disease characterized by local lymphadenopathy. Central nervous system involvement in KFD is extremely rare and remains a diagnostic challenge. Only 41 cases of aseptic meningitis associated with KFD have been reported worldwide, with just four cases (including our case) of KFD with meningitis as the first symptom. We report a case of KFD accompanied by aseptic meningitis with severely high intracranial pressure (400 mmH2O), increased white blood cell count (56 × 106/L), and moderately elevated protein level (0.52 g/L). This case is unique in the delayed appearance of lymphadenopathy. After 1 month of treatment with steroids, fever, headache, and lymphadenopathy gradually disappeared, and the result of cerebrospinal fluid examination gradually became normal. In conclusion, based on our case findings and our literature review on KFD with aseptic meningitis, a diagnosis of KFD should be considered when delayed appearance of lymphadenopathy is observed in patients with aseptic meningitis.

Aseptic meningitis in Kikuchi-Fujimoto Disease - Rare manifestation of a rare disease

“…In a more recent review by Dumas et al [ 18 ] that analyzed 91 cases diagnosed between 1989 and 2011 in 13 French hospital centers, only 2 patients (2.2%) were found to have neurological manifestations. This affirms that the association of aseptic meningitis with KFD, an already rare disease, is a rare one and very few such cases have been reported globally [ 19 ]. However, to the best of our knowledge, no such case has ever been published from Pakistan.…”

“…Most of the published literature describes cases of KFD with aseptic meningitis as either its first manifestation or during an established course of KFD illness. [ 19 , 20 ]. However, our case report is unique in the fact that our patient showed non-specific neurological findings 2–3 weeks after treatment and resolution of KFD symptoms.…”

Kikuchi–Fujimoto disease associated with the central nervous system in children

“…It is older than the mean ages in KFD pediatric studies and has ranged from 11 to 13.2 years old 19,20 . Meanwhile, in adult patients, the age range was 19–57 with a median age of 28 years old, and most of the onset age was less than 30 years (70%) 13–18 …”

“…KFD with neurological symptoms is rare. There have been 33 reported cases of KFD complicated with central nervous system injury in adults 13–18 . Only a few cases of KFD with central nervous system damage have been reported in children.…”

Unique presentation of recurrent subdural effusions and subsequent abdominal lymphadenopathy in a patient diagnosed with Kikuchi-Fujimoto disease

“…The Central Nervous System (CNS) can also be affected in very rare cases, resulting in spontaneous subdural hematomas or aseptic meningitis [9] . Few cases in the literature have been reported with manifestations of KFD in involved portions of the meninges or the CSF space, and even fewer cases with recurrent subdural fluid collections which manifest as empyema, hematomas, or effusions, as shown in the present case workup.…”