Haemophagocytic lymphohistiocytosis triggered by Kikuchi-Fujimoto disease: a diagnostic challenge.
Tanya Manish Arickatt, Fatima Memon, Anagha Harikumar et al.
✦ AI-curated · Sources linked
Case in brief
A man in his 40s presented with fever, confusion, a widespread erythematous rash, weight loss, and generalized lymphadenopathy over three weeks. He was diagnosed with secondary haemophagocytic lymphohistiocytosis (HLH) based on established criteria. Following a lymph node biopsy, Kikuchi-Fujimoto disease was identified as the underlying cause. The patient improved significantly after treatment with anakinra and dexamethasone.
What made this case unusual
This case highlights the rare association between Kikuchi-Fujimoto disease, typically benign, and secondary HLH, complicating the diagnostic process.
Diagnostic / clinical pearl
Clinicians should consider Kikuchi-Fujimoto disease in patients presenting with fever of unknown origin and lymphadenopathy, as it can trigger secondary HLH.
Why it matters
The overlapping clinical and radiological features of Kikuchi-Fujimoto disease and HLH can pose significant diagnostic challenges, making early recognition crucial for improving patient outcomes.