Kikuchi-Fujimoto disease (KFD), also called histiocytic necrotizing lymphadenitis, is a rare condition that was first described in Japan 24 years ago. Clinically, it presents with cervical adenopathy that may or may not be painful. The patients are predominantly of Asian ancestry and female. This adenopathy can be associated with a prodromal upper respiratory illness to include fever and malaise. Occasionally, hepatosplenomegaly and weight loss are seen. Symptoms usually resolve 1 to 4 months after onset. However, if the disease is multicentric and widespread, death can occur.In the literature KFD has been associated with the Epstein-Barr virus, herpes virus, and systemic lupus erythematosus. Its etiology, however, remains unclear. Histologically, KFD patterns its clinical course in three stages. It begins with a proliferative histiocytic phase, evolves into frank necrosis and karyolysis, and then resolves. Unfortunately, during its proliferative phase KFD can be misdiagnosed as malignant lymphoma.A recent case of KFD in a young woman is presented and used to discuss the clinical course and histopathologic and radiologic workup needed to prevent misdiagnosis. An understanding of KFD is important for the otolaryngologist treating patients with cervical adenopathy.
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