A familial case of Kikuchi-Fujimoto disease in dizygotic twins

Abstract

Background: Kikuchi-Fujimoto disease (KFD) or necrotizing histiocytic lymphadenitis, was described separately by both Kikuchi and Fujimoto in Japan in the early 1970's. Despite its rarity in the pediatric population, it is an important differential in persistent lymphadenopathy. Familial cases of KFD in the literature are rare. Here we describe the first reported case of KFD in non-identical twin sisters. Case presentation: Twin 1 presented with a 3-week history of worsening right-sided cervical lymphadenopathy, daily fevers, significant lethargy, weight loss and arthralgia of her knees and ankles at the age of 12 years in 2015. She had had an unremarkable medical history. A biopsy of her lymph nodes showed histiocytic necrosis consistent with KFD. Twin 2 presented with a three-week history of lethargy, fatigue, weight loss and left-sided posterior cervical chain lymphadenopathy at 16 years of age in 2018. She had a history of frequently relapsing nephrotic syndrome and celiac disease. A biopsy of her lymph nodes was undertaken and showed histiocytic necrosis consistent with KFD. Conclusions: KFD is a rare but self-limiting pathological process of necrotizing histiocytic lymphadenitis. Although further research is needed, there is an increasing amount of evidence which suggests a multifactorial pathological basis of disease. The two cases we document here are the first reported cases of familial KFD in dizygotic HLAidentical twins which reinforces the likely HLA-linkage in the etiology of KFD.

Recurrent Kikuchi Fujimoto Disease with Aseptic Meningitis and Familial Occurrence: A Rare Presentation

“…Our patient was initially treated with NSAIDS, but due to poor response steroids was started which showed a dramatic response. KFD occurrence among family members is reported very rarely in literature [8]. Our patient's sister has a history of Kikuchi lymphadenitis without a history of recurrence.…”

“…Recurrent KFD is a rare entity with a rate of 3-4% [7]. Familial cases of KFD are rarely reported in the literature [8].…”

Kikuchi-Fujimoto Disease in Human Leukocyte Antigen Partially Matched Siblings: A Case Study of Familial Susceptibility

“…Further suggesting a genetic association, their mother experienced similar self-resolving lymphadenitis in adolescence, although her HLA typing was not performed. Sibling cases of KFD have occasionally been reported, some with complete or partial HLA matching [ 11 - 13 ]. However, no common HLA type has been found in these case reports, probably due to the limited number of cases.…”

Kikuchi-Fujimoto disease: A comprehensive review

“…No genetic transmission or inheritance of a definite pattern has been identified despite being reported uncommonly to affect twins and even human leukocyte antigen (HLA)-identical non-twin siblings[ 58 - 61 ]. Some of these familial cases were from the common living environment and developed symptoms almost simultaneously and demonstrated serological evidence for the same infection.…”