Dr.SreeLekshmys, Dr.C.Jayakumar, DrSuma Balan Dr.Praveena Bhaskaran Dr.Navya George
10yr old boy presented with high grade intermittent fever of two months and was on treatment from other hospitals .He was febrile but with other stable vitals .Multiple bilateral tender cervical lymphadenopathy, largest measuring 2×1 c/m was palpated in the cervical region . Systemic examination was unremarkable. CBC and peripheral smear showed leucopenia with thrombocytopenia. CRP, ESR, LDH, ferritin, fibrinogen were elevated.
Differentials considered were:
IMN,
Brucellosis,
Toxoplasmosis,
Leukaemia and lymphoma.
IgM EBV, Brucella, Toxoplasma were negative.
USG Abdomen showed cluster of calcifications in segment VII of liver, likely healed granuloma. Retinal examination showed no evidence of uveitis or vasculitis. ANA IFA, lymphnode biopsy-was also done ANA IFA was grade 1 positive, fine speckled pattern.
In view of bicytopenia and lymphadenopathy BM aspiration biopsy done, which showed aparticulate hemodilute aspirate and paucicellular smear showing trilineage maturation and biopsy showed subcortical hypocellular marrow with trilineage maturartion. Lymphnode biopsy, showed Necrotising lymphadenitis and the possibility of Kikuchi lymphadenitis was considered.
Child was started on Naproxen, after which fever subsided. Plan is to taper and stop Naproxen. Child is now under follow up of pediatrics and pediatric Rheumatology.
Discussion:
Kikuchi Fujimoto disease (KFD) or histiocytic necrotizing lympadenitis is a rare benign self limited inflammatory disease. The etiology is unknown, certain causative organisms have been proposed include EBV, human Tcell leukemia virus type 1, human herpes virus 6, parvovirus B19, brucella, yersinia. Commonly seen in young women with localized lymphadenopathy, most commonly in the cervical region. The typical presentation is acute to subacute painful,tender,mobile cervical lymphadenopathy associated with systemic symptoms including fever, malaise, weight loss and arthralgias and various skin manifestation. An excisional lymphnode biopsy is imperative for confirming a definitive diagnosis. The characteristic histology is single or multiple areas within the lymphnode that contain necrosis and histiocytic cellular infiltrate. The capsule of the node may be invaded and perinodal inflammation is common.
No effective treatment has been established, usually a self limiting disease that resolves in 1-4 months. Management involves supportive care with corticosteroids and immunosuppresants reserved for cases of severe or recurrent disease.
Prognosis is excellent, except for relapse in some and with rare complication of HLH. Some studies indicate development of SLE months or years after diagnosis of KFD.
Carry home message
Never hesitate to get a tissue diagnosis when serology don’t help us