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Background: Kikuchi-Fujimoto disease (KFD) is a benign, self-limited, rare disease that rarely coexists with hemophagocytic lymphohistiocytosis (HLH).
Methods: We reported a case of a young female who presented with recurrent fever accompanied by painful lymphadenopathy, joint pain, and skin rash.
Results: Excisional biopsy of lymph node indicated KFD. The clinical symptoms, along with the laboratory findings met the diagnostic criteria for HLH. The patient recovered after receiving glucocorticoid and supportive care.
Conclusions: The combination of KFD and HLH exhibits distinct clinical characteristics and prognosis. Clinicians should be aware of this condition and avoid inappropriate diagnostic and therapeutic interventions.
DOI: 10.7754/Clin.Lab.2025.250227
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