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.

Pathological Features and Clinical Characteristics of Kikuchi-Fujimoto Disease: A Tertiary Hospital Experience in Riyadh, Saudi Arabia

“…Radiological investigations of KFD by positron emission tomography/computed tomography (PET/CT) showed increased metabolic activity similar to that seen in malignant diseases such as lymphoma [26,27].…”

“…The necessity for lymph node biopsy increases as misdiagnosis is being observed in up to 40% of cases by the majority of healthcare professionals on a wide range [27,28]. The pathological evaluation shows variable histologic features corresponding to the type of the disease.…”

“…Type 1 (proliferative) remains the most common type followed by the necrotic type while the xanthomatous type is less frequently seen. The variable morphological pattern especially with the presence of large lymphoid cells, immunoblasts, plasmacytoid dendritic cells, apoptosis, and necrosis increases the risk of misdiagnosis as lymphoma [27][28][29][30]. Careful morphological evaluation with the use of appropriate immunohistochemical stains can help in avoiding this problem.…”