Kikuchi-Fujimoto Syndrome: A Rare Entity to Consider

Area of Science:

• Pathology
• Immunology
• Infectious Diseases

Background:

• Kikuchi-Fujimoto disease (KFD) is a rare, benign necrotizing lymphadenitis of unknown cause.
• It typically presents with cervical lymphadenopathy, fever, and nocturnal diaphoresis.
• Diagnosis relies on histopathological study and immunophenotyping of lymph node biopsies.

Purpose of the Study:

• To highlight the diverse clinical presentations of KFD.
• To emphasize the importance of considering KFD in the differential diagnosis of fever of unknown origin.
• To discuss the potential for KFD to evolve into autoimmune disorders.

Main Methods:

• Case series describing five patients with KFD.
• Inclusion of patients with atypical presentations and varied etiologies.
• Review of diagnostic challenges and treatment implications.

Main Results:

• KFD can present atypically, mimicking conditions like pyelonephritis.
• Misdiagnosis leading to unnecessary chemotherapy occurred in some cases.
• Cytomegalovirus was identified as a potential etiological agent in one case.
• One patient developed an autoimmune condition following KFD.

Conclusions:

• KFD is a rare condition that can mimic infectious, autoimmune, and neoplastic diseases.
• It requires inclusion in the differential diagnosis for unexplained cervical lymphadenopathy and fever.
• While often self-limiting, KFD carries a risk of subsequent autoimmune syndrome development.