Kikuchi-Fujimoto disease: a case report and the evaluation of diagnostic procedures

Abstract

Background: Kikuchi-Fujimoto disease, known as histiocytic necrotizing lymphadenitis, is a benign, self-limiting and systemic disorder involving lymph nodes with unknown aetiology. First reported in Japan, there has been an increase in its incidence globally. Because of its non-specific clinical features, the disease can be easily mistaken for other forms of lymphadenitis with a high rate of misdiagnosis and mistreatment, posing a considerable challenge.

Case presentation: A case of young Chinese woman with fever and cervical lymphadenopathy is presented. Physical examinations and imaging techniques were used to rule out common forms of lymphadenitis (e.g. cat-scratch disease and tuberculous lymphadenitis). Laboratory tests were then conducted to exclude others such as systemic lupus erythematosus and non-Hodgkin lymphoma. After Kikuchi-Fujimoto disease was identified, the patient was managed with symptomatic treatments. Our case was compared with relevant cases in the literature. A diagnostic flow chart was proposed to facilitate the diagnosis and treatment.

Conclusions: With its shared clinical features, Kikuchi-Fujimoto disease can be mistaken for other forms of lymphadenitis. A combined use of medical imaging and laboratory tests is the effective way to avoid misdiagnosis.

Keywords: Diagnosis; Histiocytic necrotizing lymphadenitis; Kikuchi-Fujimoto disease; Rare disease.

Fig. 1

a Photomicrograph (magnified × 40) of paracortical area of the lymph node from the patient’s left neck; b an enlarged part, showing the characteristic features of HNL with multifocal coagulative necrosis: plentiful nuclear debris (arrowed in Blue), large mononuclear cells in the periphery (arrowed in RED), and no visible neutrophil in the necrotic area

Fig. 2

A flow chart for identifying KFD (Kikuchi-Fujimoto Disease) from other forms of cervical lymphadenitis. WS Stain: Warthin-Starry stain; PPD test: Purified protein derivative test; ANA: Anti-nuclear antibody; MDT-FC: Molecular diagnostic test and flow cytometry; MPO/CD68: myeloperoxidase/cluster of differentiation 68 protein. CSD: Cat-scratch disease; TbL: Tuberculous lymphadenitis; SLE: Systemic lupus erythematosus; NHL: Non-Hodgkin’s Lymphoma. KFD: Kikuchi-Fujimoto disease