Kikuchi-Fujimoto Disease in Michigan: A Rare Case Report and Review of the Literature

Abstract

Background: Kikuchi-Fujimoto Disease (KFD), also known as Histiocytic Necrotizing Lymphadenitis, is a rare cause of prolonged cervical lymphadenopathy in both the pediatric and adult populations. It was first reported in Japan in 1972, and since, this disease has been described worldwide, although most cases have been reported in Asia. The etiology of KFD is not fully understood, although there are 2 theories that are described in detail in this review. Kikuchi-Fujimoto Disease typically follows a benign course, with resolution of the lymphadenopathy within 6 months. It is important to recognize KFD as a cause of persistent lymphadenopathy, as it shares many characteristics with and must be differentiated from other causes of lymphadenopathy, including lymphoma, inflammatory disorders, autoimmune conditions, and infectious causes of lymphadenopathy.

Case presentation: Here is presented a case of an 11-year-old male who was born in Vietnam, but subsequently adopted and raised in the United States, who presented to a private practice community-based Otolaryngology group. His chief complaint was a persistent neck mass of approximately 3 months duration. He underwent excisional biopsy for suspected lymphoma, but final pathology rendered a diagnosis of KFD.

Conclusion: The purpose of this article is not only to review the literature but also to contribute awareness of this entity in the differential diagnosis of persistent lymphadenopathy, especially for the general Otolaryngologist in a community-based setting. In addition, this review would be beneficial for other practitioners as well, specifically Pediatricians, Infectious Disease Physicians, Rheumatologists, Pathologists, and Medical Oncologists.

Keywords: Kikuchi-Fujimoto Disease; diagnosis; histiocytic necrotizing lymphadenitis; lymphadenopathy; prognosis; systemic lupus erythematosus; treatment.