Un état de mal épileptique révélant la maladie de Kikuchi-Fujimoto : à propos d’un cas et revue de la littérature

Kikuchi-Fujimoto disease (KFD) is a rare form of lymphadenitis that primarily affects young women in Japan and other parts of Asia. It is caused by unknown agents, possibly viruses that trigger an autoimmune response in genetically susceptible individuals. KFD is often misdiagnosed due to its resemblance to other benign and malignant conditions, including SLE-related lymphadenitis and lymphoma. Diagnosis of KFD requires a lymph node biopsy, which reveals characteristic features such as histiocytic necrotizing lymphadenitis. Additional diagnostic tests, including skin biopsy, MRI, and cerebrospinal fluid analysis, can aid in confirming the diagnosis. KFD is self-limiting, and most patients recover within a few months with symptomatic treatment. However, early intervention with corticosteroids and hydroxychloroquine is crucial for effective management, particularly in cases with extra-nodal involvement or neurological symptoms. Lymph node removal can also be an effective therapeutic measure, and Hemophagocytic lymphohistiocytosis (HLH), a potential complication of KFD, is managed with a combination of immunoglobulins and steroids. While KFD is a benign condition, its diagnosis and treatment can be challenging, requiring close monitoring and follow-up.

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Kikuchi-Fujimoto Disease: A Review

Perry¹,

Choi

2018

Archives of Pathology &Amp; Laboratory Medicine

177

216

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Kikuchi-Fujimoto disease (KFD) is a rare entity characterized by subacute necrotizing lymphadenopathy and frequently associated with fever. Young adults of Asian ancestry are most commonly affected, but it has been reported worldwide. Despite many studies in the literature, the cause of KFD remains uncertain. Histologically, KFD is characterized by paracortical lymph node expansion with patchy, well-circumscribed areas of necrosis showing abundant karyorrhectic nuclear debris and absence of neutrophils and eosinophils. Three evolving histologic patterns—proliferative, necrotizing, and xanthomatous—have been recognized. By immunohistochemistry, histiocytes in KFD are positive for myeloperoxidase. There is a marked predominance of T cells in the lesions (with mostly CD8-positive cells) with very few B cells. The differential diagnosis of KFD includes infectious lymphadenitis, autoimmune lymphadenopathy (primarily systemic lupus erythematosus), and lymphoma. Clinicians and pathologists are poorly familiar with this entity, which frequently causes significant diagnostic challenges.

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Un état de mal épileptique révélant la maladie de Kikuchi-Fujimoto : à propos d’un cas et revue de la littérature

Cited by 3 publications

References 3 publications

Ankylosing spondylitis with Kikuchi–Fujimoto disease: A case report with literature review

Ankylosing spondylitis with Kikuchi–Fujimoto disease: A case report with literature review

Kikuchi-Fujimoto Disease: A Rare Lymphadenitis with Diagnostic Challenges and Treatment Considerations

Kikuchi-Fujimoto Disease: A Review

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