Kikuchi-Fujimoto disease (histiocytic necrotizing lymphadenitis) with atypical encephalitis and painful testitis: a case report

Abstract

BackgroundKikuchi-Fujimoto disease is a self-limited clinicopathologic entity that is increasingly recognized worldwide. Kikuchi-Fujimoto disease is characterized by cervical lymphadenopathy occurring in young adults. Neurologic involvement is rare, and testitis directly caused by Kikuchi-Fujimoto disease has not yet been reported.Case presentationA 19-year-old man was brought to our clinic with complaints of fever, headache, fatigue, and left lower quadrant pain that had persisted for 3 weeks. On physical examination, painful cervical lymphadenopathies were observed. Meningitis was suspected based on a cerebrospinal fluid examination, and left-sided orchitis was diagnosed based on findings from magnetic resonance imaging and ultrasonography. However, neither antibiotics nor antiviral drugs were effective in treating the patient’s symptoms. On the 20th day of hospitalization, the patient experienced a loss of consciousness, and brain T2-weighted magnetic resonance imaging showed asymmetrical, high-signal intensities in both basal nuclei and the left temporal lobe. Encephalitis was suspected, and the patient was treated with intravenous prednisolone pulse therapy (1 g/day) for 3 days and intravenous immunoglobulin therapy for 5 days. A left cervical lymph node biopsy showed apoptotic necrosis in paracortical and cortical areas with an abundance of macrophages and large lymphoid cells, which had irregular nuclei suggestive of Kikuchi-Fujimoto disease; the pathological findings from a brain biopsy were the same as those of the cervical lymph node biopsy. The encephalitis and cervical lymphadenopathies followed a benign course, as did the testitis.ConclusionsThis is the first report of Kikuchi-Fujimoto disease involving painful testitis and pathologically proven asymmetrical brain regions. Kikuchi-Fujimoto disease should be included in the differential diagnosis when a patient presents with encephalitis, testitis, and fever of unknown origin.

An atypical neurologic complication of Kikuchi-Fujimoto Disease

“…Given the recent diagnosis via cervical node biopsy, knowledge that KFD can lead to CNS involvement, improvement in the lesion without treatment, and rapid resolution, the lesion is a presumed complication of Kikuchi-Fujimoto disease. 5 The unique aspect of this single case is the resolution with supportive management because she did not receive steroids or immunomodulating therapy since the onset of her weakness. This suggests that, in some cases, the CNS lesions of KFD may resolve with supportive care.…”

Two Cases of Kikuchi Disease Presenting with Aseptic Meningitis and Encephalitis

“…Third, we encountered an unusual case of encephalitis associated with Kikuchi disease. There are few cases of encephalitis accompanied by Kikuchi disease (5)(6)(7)(8)(9). Byun et al reported the clinical and MRI findings of nine patients with encephalitis associated with Kikuchi disease (9).…”

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

“…The clinical manifestations of KFD involving the CNS are complex and diverse, including meningitis, encephalitis, subdural effusion, optic neuritis, cerebellar ataxia, hemiplegia, and other signs (10,23,(34)(35)(36)(37)(38). KFD concomitant with neurological symptoms is rare and prone to missed diagnosis and misdiagnosis.…”