2024/12/30 by Kolsi R ., Belhaj R ., Ben Ameur S . +5
#Kikuchi-Fujimoto disease #histiocytic necrotizing lymphadenitis #infant #lymphadenopathy
paper · doi:10.71566/pist-rmp-194611
Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a rare inflammatory di sorder affecting both children and adults all over the world with a higher prevalence among Asiatic po pulations. It is characterized by cervical lymphadenopathy, often associated with fever and night sweats. Its etiopathogenesis is not fully understood, although it may include predisposing genetic background and a possible infectious triggering event. The diagnosis is provided by anatomopathological and immu nohistochemical study. The clinical course is generally favorable within few months. Herein, we report a case of a Tunisian infant presented with fever and multiple cervical lymphadenopathies complicated with subcutaneous infiltration and edema. The biological tests showed pancytopenia. Within 48 hours, the pa tient showed signs of respiratory distress due to tracheal compression. He was admitted in a pediatric intensive care unit and required assisted ventilation. He was also treated with antibiotics and intravenous corticosteroids. Histopathological examination of a lymph node biopsy showed necrotizing lymphadenitis with histiocyte infiltrate and multiple apoptotic cells and allowed the diagnosis of Kikuchi-Fujimoto disease. Clinical remission was achieved within two weeks. In conclusion, this uncommon disease has a wide clinical spectrum that sometimes includes severe symptoms. Thus, clinicians and pathologists should be aware of it in order to differentiate it from infectious diseases, lymphoma and other inflammatory disorders.