Kikuchi-Fujimoto disease (KFD) is a rare and self-limiting disorder that predominantly affects young individuals of Asian descent. This case report describes familial KFD in partially human leukocyte antigen (HLA)-matched siblings. An adolescent male presented with cervical lymphadenopathy and elevated lactate dehydrogenase (LDH) levels, diagnosed by biopsy as KFD; approximately one year later, his sister presented with similar symptoms. Both siblings were found to carry the HLA-DPB1*0202 allele, which is commonly associated with KFD. These cases highlight a genetic component in KFD and encourage further genetic research to delineate the pathogenesis of the disease.
Keywords: familial susceptibility; histiocytic necrotizing lymphadenitis; human leukocyte antigen; kikuchi-fujimoto disease; sibling.
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