Kikuchi-Fujimoto Disease: Eleven Pediatric Cases and Literature Review

KFD was first described in 1972 as a case series in form of benign necrotizing lymphadenitis with distinctive histologic findings.1 The most common clinical features in KFD is prolonged fever and cervical lymphadenopathy which is in most cases unilateral and painful.2-4 Kikuchi-Fujimoto disease has a worldwide distribution with a higher prevalence among Asian people.5,6 The first pediatric case of KFD was reported in 1990, which was followed by many studies reporting considerable number of children with KFD.3,7 Diagnosis of KFD depends upon presence of appropriate histopathology of the lymph node (LN), which typically shows paracortical well-circumscribed necrotic lesions consisting of karyorrhexis, fibrin deposits, abundant CD68 plasmacytoid monocytes and infiltration of histiocytes in absence of plasma cells or neutrophils.2-5 We report eleven children with KFD in a period of 10 years. [...]autoimmune manifestations have been widely reported in KFD presentation, and in some cases, KFD preceded clinical manifestation of systemic lupus erythematosus (SLE), which raised the proposal that KFD may reflect autoimmune conditions like SLE that may be induced by virus-infected transformed lymphocytes.1,6,8,9 A comparison of our patients data with other studies discussed in the literature (Table 2) revealed that the overall clinical and laboratory results are similar. The cell death process in KFD has been proposed by some as apoptosis because of the nuclear fragments seen within and surrounding the typical area of necrosis in the affected LNs; that is consistent with the typical feature of early apoptosis.12 Moreover, CD8+T lymphocytes undergo apoptosis within the necrotized lesion in the affected LNs.12,13 Monocyte and macrophage cell lines have been suggested to promote the histiocyte-dependent CD8+T cell death.14 The most common autoimmune disease associated with KFD is SLE.