Kikuchi-Fujimoto disease: A comprehensive review

Abstract

Kikuchi-Fujimoto disease, a rare form of necrotizing lymphadenitis, is an uncommon, benign, self-limiting disorder of obscure etiology. It affects mostly young adults of both genders. Clinically, it presents with fever and lymphadenopathy of a firm to rubbery consistency frequently involving cervical lymph nodes while weight loss, splenomegaly, leucopenia, and elevated erythrocyte sedimentation rate feature in severely affected patients. Cutaneous involvement occurs in about 30%-40% of cases as facial erythema and nonspecific erythematous papules, plaques, acneiform or morbilliform lesions of great histologic heterogeneity. Both Kikuchi-Fujimoto disease and systemic lupus erythematosus share an obscure and complex relationship as systemic lupus erythematosus may occasionally precede, develop subsequently, or sometimes be associated concurrently with Kikuchi-Fujimoto disease. It is often mistaken for non-Hodgkin lymphoma while lupus lymphadenitis, cat-scratch disease, Sweet’s syndrome, Still’s disease, drug eruptions, infectious mononucleosis, and viral or tubercular lymphadenitis are other common differentials. Fine needle aspiration cytology mostly has features of nonspecific reactive lymphadenitis and immunohistochemistry studies usually show variable features of uncertain diagnostic value . Since its diagnosis is exclusively from histopathology, it needs to be evaluated more carefully; an early lymph node biopsy will obviate the need for unnecessary investigations and therapeutic trials. Its treatment with systemic corticosteroids, hydroxychloroquine, or antimicrobial agents mostly remains empirical. The article reviews clinicoepidemiological, diagnostic, and management aspects of KFD from the perspective of practicing clinicians.

Optic Neuritis in a Pediatric Patient with Kikuchi–Fujimoto Disease: A Case Report and Review of the Literature

“…However, the urgent intervention is required in systemic complications of the KFD, which involve generalized lymphadenopathy, arthralgia, rash, night sweats, weight loss, leucopenia, and neurological involvement, including aseptic meningitis, peripheral neuropathy, kinetic tremor and cerebellar ataxia. 1 While KFD is seldom associated with ocular manifestations, it has been found to present in various forms, including uveitis, retinal vein vasculitis, and optic neuritis (observed in 4 patients, including our case and another patient with neuromyelitis optica spectrum disorder, NMOSD). [2][3][4] Alongside the limited instances of optic neuritis associated with KFD in the existing literature, our case report adds to the knowledge by presenting a 13-year-old male patient with KFD and bilateral optic neuritis.…”

“…KFD, a rare condition characterized by the histiocytic necrotizing lymphadenitis, typically affects young East Asian females, with a male/female ratio of 1:1.3–4. 1 However, KFD in the pediatric population has a male predominance. A male/female ratio of 1.9:1 and a median age of 10 years in the childhood-onset KFD were previously reported in our hospital.…”

“…However, the urgent intervention is required in systemic complications of the KFD, which involve generalized lymphadenopathy, arthralgia, rash, night sweats, weight loss, leucopenia, and neurological involvement, including aseptic meningitis, peripheral neuropathy, kinetic tremor and cerebellar ataxia. 1 …”

The clinical and pathological features of Kimura disease in pediatric patients

“…CSD presents as chronic lymphadenopathy often associated with fever following bites or scratches from animals or plant materials, exhibiting central stellate microabscesses and peripheral granulomatous inflammation histologically ( 14 ). KFD is a prevalent lymphadenopathy featuring necrotic nodules comprising numerous tissue cells with round to crescent-shaped nuclei and tissue cell fragments ( 15 ), with CD123 immunohistochemical staining aiding in the differentiation of Kikuchi tissue cells ( 16 ). Parasitic infections typically manifest as an accumulation of eosinophils forming granulomas, sometimes with observable parasite eggs ( 17 ).…”

Kikuchi Fujimoto Lymphadenitis: A Rare Association With COVID-19 Vaccination

“…Kikuchi-Fujimoto disease (KFD), often called necrotizing histiocytic lymphadenitis, is primarily a rare entity characterized by lymphadenopathy and fever. It was first found in 1972 in Japan, and around 41 cases were reported until 2021 [ 1 - 3 ]. Usually, KFD is a benign, self-resolving ailment.…”