Introduction Kikuchi disease (histiocytic necrotizing lymphadenitis) first described in Japan (1972) is a self–limited disorder of unknown aetiology, characterised by focal painful lymphadenitis, fever, malaise and weight loss.1 Kikuchi disease affects a wide age range of patients (2–75 years) but typically affects young adults (mean age, 20–30 years).2 It has been reported predominantly in Asian population and occurs sporadically outside Asia.
We report a case of Kikuchi disease with multisystem involvement in a 14–year–old Chinese boy.
Case report Child was admitted to our hospital with 3 weeks of fever (daily spikes > 39°C), intermittent frontal headaches, rash, weight loss (2 kg) and oral ulcers for one week. No travel history.
Clinical examination: Febrile child with left cervical and left submandibular lymphadenopathy, hepatomegaly and maculopapular rash. Rest of the examination was unremarkable.
Child was investigated for fever of unknown origin and treated with intravenous antibiotics (ceftriaxone and clindamycin).
Investigations: Blood, stool, urine cultures, Rickettsia, Toxoplasmosis, EBV, CMV, Salmonella serology, HSV PCR, TB (T –spot test), ANA and anti dsDNA negative. Raised ASOT (800 units), LDH 524 (170–283 U/L), ESR (170 mm/hr) and CRP (46 mg/l). Bone Marrow showed no evidence of malignancy. Lymph node biopsy was consistent with Kikuchi’s lymphadenitis.
Child was discharged after 12 days with oral prednisolone but readmitted 4 days later with seizures requiring anticonvulsants and ventilation. Child was diagnosed with Kikuchi’s disease with multisystem involvement (aseptic meningitis, raised liver transaminases, DCT positive anaemia, and interface dermatitis). MRI/MRA brain was normal. CSF cultures were negative. Child was treated with intravenous antibiotics and methylprednisolone followed by oral steroids and antibiotics on discharge. Child has remained well on follow up clinic visits.
Conclusions Clinical differential diagnosis for Kikuchi disease includes infectious mononucleosis, bacterial lymphadenitis, cat scratch disease, mycobacterium tuberculosis, CMV disease, toxoplasmosis, systemic lupus erythematosus (SLE), malignancy and kawasaki disease.1
Diagnosis of Kikuchi’s disease is confirmed by lymph node biopsy showing histiocytic necrotizing lymphadenitis (similar to SLE); however, association of Kikuchi disease with SLE remains unclear. Corticosteroid therapy may speed up recovery in patients with kikuchi disease1. The prognosis for kikuchi is generally optimistic; however, a concurrent autoimmune disease or the risk of developing an autoimmune disease needs careful monitoring.3
Feder HM, Liu J, Rezuke WN. Kikuchi disease in connecticut. J Pediatr. 2014;164:196–200
Chaitanya BN, Sindura CS. Kikuchi’s disease. J Oral Maxillofac Pathol. 2010;14(1):6–9
Cheng CY, Sheng WH, Lo YC, et alet al. Clinical presentations, laboratory results and outcomes of patients with Kikuchi’s disease. J Microbiol Immunol Infect. 2010;43(5):366–71
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