Kikuchi-Fujimoto disease: a sheep in wolf's clothing

J Otolaryngol Head Neck Surg. 2012 Jun 1;41(3):222-6.

Abstract

Introduction: Kikuchi-Fujimoto disease (KFD) is a rare, self-limiting disorder that typically affects the cervical lymph nodes. Recognition of this condition is crucial, especially because it can easily be mistaken for tuberculosis, lymphoma, or even adenocarcinoma. Awareness of this disorder will help prevent misdiagnosis and inappropriate treatment.

Methods: From January 2006 to December 2008, 30 patients who underwent a biopsy of a cervical lymph node and proved histologically to have KFD were enrolled in this study. We studied clinical manifestations, laboratory results, treatment, and recurrence for each patient. Patients were followed up for a mean period of 2 years.

Results: There were 24 women and 6 men, with a mean age of 18 years. Two patients had a past history of tuberculosis. Major clinical symptoms and signs were fever (70%) and lymphadenopathy (100%). The affected cervical lymph nodes were most commonly located in the posterior cervical triangle. Unilateral and bilateral cervical lymph nodes were affected in 25 and 5 patients, respectively. The affected lymph nodes were most commonly less than 3 cm in size. Leukopenia was observed in 46.7%, and a raised erythrocyte sedimentation rate was seen in 56.7% of the cases. Treatment strategies included no medication, nonsteroidal antiinflammatory drugs (NSAIDs) alone, steroids alone, or a combination of NSAIDs and steroids. Ninety percent improved within 3 months, whereas one patient showed improvement only after 9 months of continued treatment. No recurrence has since been noted.

Conclusion: KFD is a benign disease that masquerades as other more sinister diseases and can lead to unnecessary treatment-induced physiologic, psychological, and financial morbidity to the patient. Tissue diagnosis is necessary in all cases, and an effective communication between the surgeon and the pathologist is imperative in making an accurate diagnosis.

MeSH terms

  • Adolescent
  • Adult
  • Biopsy
  • Child
  • Diagnosis, Differential
  • Female
  • Histiocytic Necrotizing Lymphadenitis / diagnosis*
  • Histiocytic Necrotizing Lymphadenitis / drug therapy
  • Histiocytic Necrotizing Lymphadenitis / pathology
  • Humans
  • Male
  • Retrospective Studies