Diffuse Lymphadenopathy and Prolonged Fever as a Severe form of Kikuchi Disease: A Case Report

Background: Kikuchi-Fujimoto disease, is a benign, self-limited disease that is primarily characterized with cervical lymphadenopathy and fever. Its presentation frequently requires a broad diagnostic workup including extensive laboratorial exams and imaging studies as needed in the evaluation of lymphoproliferative syndromes. Due to its rarity and nonspecific clinical features, Kikuchi disease might be missed or otherwise misdiagnosed and unnecessarily treated with antibiotics, steroids and even chemotherapy.

Case Presentation: We report of a 17 years-old adolescent presenting with 4 weeks of fever, diffuse lymphadenopathy, arthralgia and a skin rash. Her preadmission laboratorial exams revealed worrisome results of leukopenia, anemia and increased lactate dehydrogenase (LDH) levels. Managed as a case of fever without source at presentation, she completed a thorough investigation considering infectious, inflammatory and neoplastic etiologies. In light of a suspected diagnosis of lymphoma she completed a positron emission tomography (PET) scan with increased uptake on both sides of the diaphragm. A needle biopsy of an axillary lymph node was unable to determine a diagnosis while the patient showed clinical deterioration and increasing levels of C-reactive protein. Under Naproxen treatment, the patient completed an excisional lymph node biopsy confirming the diagnosis of Kikuchi disease. She showed a spontaneous gradual recovery soon thereafter.

Conclusions: Despite its benign nature, Kikuchi disease might have a severe form of clinical and laboratorial presentation. The diagnosis is exclusively made by a lymph node biopsy, which should be considered early in the investigational workup if a neoplastic etiology is suspected.