Kikuchi-Fujimoto Syndrome: A Rare Entity to Consider

Authors

• Diogo Raposo André Internal Medicine Department, Hospital Central do Funchal, Madeira Island, Portugal
• Filipa Vicente Internal Medicine Department, Hospital Central do Funchal, Madeira Island, Portugal
• Jessica Chaves Internal Medicine Department, Hospital Central do Funchal, Madeira Island, Portugal
• Mónica Caldeira Internal Medicine Department, Hospital Central do Funchal, Madeira Island, Portugal
• Fernando Jacinto Internal Medicine Department, Hospital Central do Funchal, Madeira Island, Portugal
• António José Chaves Internal Medicine Department, Hospital Central do Funchal, Madeira Island, Portugal
• Maria Luz Brazão Internal Medicine Department, Hospital Central do Funchal, Madeira Island, Portugal

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Keywords

Abstract

Introduction: Kikuchi-Fujimoto disease (KFD) is a rare, benign, necrotizing lymphadenitis of unknown aetiology with good prognosis. It is characterized by cervical lymphadenopathy, nocturnal diaphoresis and fever. Surgical excision of the adenopathy, histopathological study and immunophenotyping are crucial for diagnosis.
Patients and methods: This paper describes five patients with three different histological subtypes of KFD, including an atypical presentation masquerading as pyelonephritis and two other cases where physicians mistakenly started chemotherapy. In one other case cytomegalovirus was identified as the responsible aetiological agent, while in the remaining patient, KFD evolved into an autoimmune condition.
Discussion: KFD, although rare, may mimic infectious, autoimmune and neoplastic diseases. It also poses a risk for the subsequent development of an autoimmune disorder.