Kikuchi Fujimoto as an Initial Presentation of Systemic Lupus Erythromatosis

Laxman Shrestha,Prabina Shrestha,Srijana Basnet

doi:10.3126/jnps.v41i1.29519

Laxman Shrestha, Prabina Shrestha + Show 1 more

Open Access

https://doi.org/10.3126/jnps.v41i1.29519

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Abstract

    Kikuchi-Fujimoto Disease (KFD) is a rare benign, condition of necrotising histiocytic lymphadenitis. In this case report, we discuss a case of 10 year old male patient who presented with a fever, rash and generalised lymphadenopathy that was not attributable to the more common causes. Axillary lymph node biopsy confirmed the diagnosis of KFD. Treatment with prednisolone improved his symptoms but after six months he had recurrence of his symptoms. He was investigated again and finally met diagnostic criteria for SLE. This case report highlights importance of close follow up in a child with KFD.   

Highlights

Kikuchi–Fujimoto disease (KFD) is a rare benign disease with clinical presentation of fever and cervical lymphadenopathy
A limited number of cases are found in the literature showing an association between KFD and SLE in male patients
Initial clinical presentation of this case resembles that of infectious origin like viral or rickettsial infection

Summary

INTRODUCTION

Kikuchi–Fujimoto disease (KFD) is a rare benign disease with clinical presentation of fever and cervical lymphadenopathy This condition is confirmed histologically by histiocytic necrotising lymphadenitis without granulocytic infiltration. Multiple erythematous papular rashes noted over face and over trunk which were crusted with central scab formation and turned to perilesional hypopigmented area, over five days. Findings were consistent with necrotising histiocytic lymphadenitis (Kikuchi lymphadenitis) He was given prednisolone 1 mg/kg/d for two weeks. Steroid was gradually tapered and stopped over two weeks After six months, he again developed fever and rashes. His ANA and dsDNA were sent which were positive He was diagnosed as SLE and started on prednisolone. The boy was started on hydroxychloroquine and advised for use of sunscreen

DISCUSSION

Findings

CONCLUSIONS