Kikuchi-Fujimoto Disease

Report of Clinical Case

  • Claudia Llorente-Álvarez
    Hospital Universitario Río Hortega https://orcid.org/0000-0001-9108-6898 cllorentea[at]saludcastillayleon.es
  • Laura Manuela Bayona-Romero
    Hospital Universitario Río Hortega
  • Carmen Vallés-Rodríguez
    Hospital Universitario Río Hortega
  • Isidora Paz Rettig-Infante
    Hospital Universitario Río Hortega
  • Manuel Acuña-García
    Hospital Universitario Río Hortega
  • María Miguel Lucero-Salaverry
    Hospital Universitario Río Hortega
  • María Ángeles Torres-Nieto
    Hospital Universitario Río Hortega

Abstract

Introduction and objective: Kikuchi-Fujimoto disease (KDF) or non-granulocytic necrotizing lymphadenitis was first described in 1972 in young Japanese women; however, cases have now been observed in different ethnic groups and countries. The etiology is unknown, although it is postulated that it could have an autoimmune origin or due to viral infections, results of studies on viral etiology being inconsistent.Method: A 41-year-old patient presented with 2 painful right lateral cervical lymphadenopathy, that progressively increased in size. The patient presented no fever, night sweats, or associated weight loss. No other symptoms appeared.Results: On examination, right lateral cervical lymphadenopathy of 1.5cm were palpated, soft, mobile and not adhered, with the rest of the examination being normal. Blood tests were performed, including a proteinogram, autoimmunity study, and serology for HBV, HCV, HIV, CMV, VZV, EBV, Rubella, Toxoplasma, and Treponema pallidum. All of them with negative results. It was decided to perform an ultrasound with FNA of the same with the result compatible with necrotizing histiocytic lymphadenitis or Kikuchi lymphadenitis. It was decided to perform exeresis of the lymphadenopathy, confirming the diagnosis and subsequent conservative treatment.Discussion: KFD is a benign and self-limited form of necrotizing lymphadenitis, with a recurrence of 15% up to 8 years after the initial symptoms. Between 60-90% of patients have posterocervical lymph nodes that are painful on palpation, which can be associated with systemic B symptoms. In the differential diagnosis we must include lymphadenitis of infectious origin, lymphomas and autoimmune diseases such as systemic lupus erythematosus. The definitive diagnosis is the histological examination of adenopathy, in which reactive hyperplasia is observed with foci of paracortical necrosis with cellular debris, surrounded by an inflammatory infiltrate of CD4+ lymphocytes, CD68 histiocytes, and scarce presence of polymorphonuclear neutrophils and plasma cells. The most common treatment is observation, and highly symptomatic patients may benefit from the administration of corticosteroids or non-steroidal anti-inflammatory drugs.Conclusions: EKF should be included in the differential diagnosis of a patient presenting with cervical lymphadenopathy and fever of unknown origin. Despite its self-limited nature and complete resolution in most cases, there is a risk of progression to autoimmune disease or recurrence. The definitive diagnosis is histological examination.
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Llorente-Álvarez, C., Bayona-Romero, L. M., Vallés-Rodríguez, C., Rettig-Infante, I. P., Acuña-García, M., Lucero-Salaverry, M. M., & Torres-Nieto, M. Ángeles. (2023). Kikuchi-Fujimoto Disease: Report of Clinical Case. Revista ORL, 13(S2), 161–162. https://doi.org/10.14201/orl.29046

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