• J Coll Physicians Surg Pak · Jun 2018

    Case Reports

    A Rare Case of Kikuchi-Fujimoto Disease Associated with Systemic Lupus Erythematosus.

    • Attia Bari, Fatima Zeeshan, Ghazala Hanif, Uzma Jabeen, Iqbal Bano, and Ahsan Waheed Rathore.
    • Department of Pediatric Medicine, The Children's Hospital and the ICH, Lahore.
    • J Coll Physicians Surg Pak. 2018 Jun 1; 28 (6): S143-S145.

    AbstractKikuchi-Fujimoto Disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a self-remitting, immune-mediated rare disorder having unique histopathological characteristics which is commonly seen in young Asian females, but can occur in all ethnicities. There is a strong association between KFD and Systemic Lupus Erythematosus (SLE). We present a case of a young Pakistani boy who presented with cervical lymphadenopathy, fever, blackish discoloration of finger tips, and Raynaud's phenomenon. His lymph node biopsy was suggestive of KFD. The American Rheumatology Association diagnostic criteria were not met as no other features of SLE were present. His autoimmune workup including Anti-Nuclear Antibodies (ANA) and Anti-Double Stranded DNA (Anti-Ds DNA) antibodies were positive and supported the diagnosis of SLE. He improved clinically with steroid therapy and nifedipine with resolution of symptoms.

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