BMJ Case Reports 2018; doi:10.1136/bcr-2018-225668
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Histiocytic necrotising lymphadenitis identical to Kikuchi-Fujimoto disease in CNS lupus

  1. Annerose Mengel1
  1. 1 Department of Neurology and Stroke, and Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany
  2. 2 Department of Clinical Pathology, Robert-Bosch Krankenhaus, and Margarete Fischer-Bosch Institute for Clinical Pharmacology, Stuttgart, Germany
  1. Correspondence to Dr Maria Ioanna Stefanou, maria-ioanna.stefanou{at}
  • Accepted 25 May 2018
  • Published 8 June 2018


A 36-year-old woman presented after a first generalised tonic–clonic seizure. On clinical examination, prominent cervical and axillary lymphadenopathy was noted. Brain MRI showed diffuse, partially enhancing, hyperintense lesions (figure 1). An axillary lymph node biopsy revealed typical findings of histiocytic necrotising lymphadenitis, compatible with Kikuchi-Fujimoto disease (figure 2).1 Laboratory studies showed positive antinuclear antibodies, low complement levels and pancytopenia. In the cerebrospinal fluid (CSF), lymphocytic pleocytosis (34/μL), elevated lactate (3.3 mmol/L) and protein (104 mg/dL) levels were noted. Investigations for lymphoma were negative. Furthermore, negative serological and PCR findings for Epstein-Barr virus, cytomegalovirus and HIV were found. Interferon-gamma release assay and PCR for tuberculosis, along with serological investigations for toxoplasmosis and syphilis, were negative. The cytological …

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