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.
Highlights
Histiocytic necrotic lymphadenitis (HNL), first described by Japanese pathologists Kikuchi and Fujimoto et al [1, 2] and called Kikuchi–Fujimoto disease (KFD), is a rare local lymphadenopathy with a benign course and with clinical manifestations including fever, lymphadenopathy, rash, hepatosplenomegaly, central nervous system (CNS) symptoms, and hemophilic cell syndrome
Based on the clinical manifestations and the cerebrospinal fluid (CSF) and cervical lymph node biopsy findings, the patient was diagnosed with KFD with aseptic meningitis
The CSF culture was sterile, and lymph node biopsy revealed typical karyorrhectic debris and CD68+ histiocytes, which confirmed the diagnosis of KFD concurrent with aseptic meningitis
Summary
Histiocytic necrotic lymphadenitis (HNL), first described by Japanese pathologists Kikuchi and Fujimoto et al [1, 2] and called Kikuchi–Fujimoto disease (KFD), is a rare local lymphadenopathy with a benign course and with clinical manifestations including fever, lymphadenopathy, rash, hepatosplenomegaly, central nervous system (CNS) symptoms, and hemophilic cell syndrome. Biochemical detection of cerebrospinal fluid (CSF) including protein, glucose, chlorine, and white blood cells showed that these were all within normal ranges. Results of polymerase chain reaction (PCR) for herpes simplex virus, cytomegalovirus, human herpesvirus, EBV, and herpes zoster virus were normal He was administered IV with acyclovir, mannitol, and other symptomatic treatments. A third lumbar puncture examination (Table 1, day 17) showed a 400-mmH2O-high cranial pressure with elevated protein (0.52 g/L; normal range, 0.15–0.45 g/L) and white blood cell count (56 × 106/L; normal range, 0–8 × 106/L), among which monocytes represented about 92%, while glucose and chlorine levels were normal. Based on the clinical manifestations and the CSF and cervical lymph node biopsy findings, the patient was diagnosed with KFD with aseptic meningitis. After 10 months of follow-up, no symptoms or signs of meningitis relapse or evolution into other autoimmune diseases were detected
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