Aseptic meningitis revealing isolated Kikuchi-Fujimoto disease

Introduction: Kikuchi-Fujimoto’s disease (KFD) or histiocytic necrotizing lymphadenitis is a rare entity that may represent a real diagnostic challenge for the clinician because of its highly polymorphous and sometimes unusual presentations. We report an original observation of KFD with aseptic meningitis as inaugural manifestation. Case report: 30-year-old woman, without pathological medical history, was hospitalized via the emergency department for exploration of a meningeal syndrome with cervical lymphadenopathies. The lumbar puncture showed aseptic meningitis: clear, normotensive cerebrospinal fluid with leukocytes at 28/mm3 (90% lymphocytes), red blood cells at 2/mm3, proteinrrhachia at 0.58 g/l, glucorrachia at 4 mmol/l for venous glycemia at 8 mmol/l, and negative direct examination and culture. Cerebromedullary MRI and cerebral angio-MR were without abnormalities. Further infectious and immunological investigations were negative. Cervical lymph node biopsy showed histological and Immunohistochemical aspects suggesting KFD. Treated with systemic corticosteroids, the evolution was favorable with no recurrence. Conclusion: KFD-associated aseptic meningitis remains rare, and the inaugural forms are exceptional and often difficult to diagnose. A better knowledge of this association avoids unnecessary investigations, recurrence, and improves the prognosis of the disease.