Kikuchi-Fujimoto disease: the quandary continues

Abstract

Kikuchi-Fujimoto disease (KFD) is a rare lymphohistiocytic disorder with an unknown etiopathogenesis. Due to its non-specific lymphadenopathy presentation, treatment is complicated by the frequency by which it is misdiagnosed—for example up to one-third of cases are misdiagnosed as malignant lymphoma, leading to expensive clinical testing and overtreatment of this typically self-limiting illness. KFD has a strong association with SLE, although its transience and rarity make it difficult to investigate. We present a case of KFD to illustrate the variance in presentation and typical outcome of KFD. We want to increase awareness and shed some light on some typical and atypical clinical presentations of KFD to reduce the incidence of misdiagnosis.