Kikuchi–Fujimoto disease: A rare cause of lymphadenopathy and fever

Abstract

Introduction: Kikuchi–Fujimoto disease (KFD) was originally described in a young woman and is a rare, benign and self-limited condition of unknown cause, usually characterized by cervical lymphadenopathy and fever. The diagnosis of KFD is made by lymph node biopsy and treatment is only symptomatic, with signs and symptoms usually resolving within one to six months. Case Report: A 31-year-old female, rabbit breeder, presented with complaints of fever, facial and hand skin lesions for 10 days. Physical examination revealed nose, malar, scalp, fingers and pinna erythematous papules; also painful bilateral cervical and axillary lymphadenopathy. Laboratory studies showed normocytic/normochromic anemia (hemoglobin 10.4 g/dL), leukopenia (white blood cell count 2.2x103 u/L), neutropenia 44.4% and elevated erythrocyte sedimentation rate 31 mm/h. Serology for infectious agents, microbiology and autoimmune blood work studies were negative. Computed tomography scan of the neck revealed small cervical lymphadenopathy and enlarged axillary lymphadenopathy. She underwent biopsy of a right axillary lymph node, which revealed Kikuchi lymphadenitis xanthomatous type. Skin biopsy showed unspecified perivascular lymphocytic infiltrate. On a three-month follow-up visit, she continued to be afebrile, had improved white cell blood count and had only small right axillary lymphadenopathy visible on ultrasound. Skin lesions had disappeared. Conclusion: Kikuchi-Fujimoto disease should be considered in the differential diagnosis when a young woman presents with fever and cervical lymphadenopathy. Despite its benign and self-limited nature, patients should be followed for a few years since recurrence is common and some patients may develop autoimmune disorders.