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Kikuchi-Fujimoto's disease (KFD) is a rare, benign condition characterized by histiocytic necrotizing lymphadenitis predominantly in the cervical region and prolonged fever. It has a higher prevalence in the Japanese and Asian populations although it may be seen in a wide geographic distribution, but rare are the cases reported in Europe. While symptoms resolve spontaneously between 2 weeks and 2 months, complications are described in: CNS, eyes, respiratory tract, kidney and liver. KFD also described an association with systemic lupus erythematosus. This paper describes a rare case of Kikuchi-Fujimoto lymphadenitis and discusses the features of the disease. A 24-year-old man presented with fever and a swollen 3-cm lymph node in the left side of the neck. The definitive diagnosis was established, after excision of level VB nodes on the left, by histologic examination. It revealed structurally intact tissue with scalloping in the paracortical area presenting activated T cells, focal monocytoid B cells, sinus histiocytosis and normally polarized follicles. Other two samples presented focal alterations of the tissue structure due to broad areas of necrosis, conspicuous nuclear debris, large lymphoid cells as well as numerous histiocytes. Immunohistochemistry revealed CD3+ and CD8+ T lymphocytes and histiocytes expressing CD68/PGM-1 (specific for macrophage lineage) and myeloperoxidase (MPO, specific for myeloid lineage). Correct, prompt diagnosis should be established through the findings of imaging and pathologic studies to avoid unnecessary investigation and ineffective therapies.
Kikuchi-Fujimoto's disease (KFD) is a rare, benign condition characterized by histiocytic necrotizing lymphadenitis predominantly in the cervical region and prolonged fever. It has a higher prevalence in the Japanese and Asian populations although it may be seen in a wide geographic distribution, but rare are the cases reported in Europe. While symptoms resolve spontaneously between 2 weeks and 2 months, complications are described in: CNS, eyes, respiratory tract, kidney and liver. KFD also described an association with systemic lupus erythematosus. This paper describes a rare case of Kikuchi-Fujimoto lymphadenitis and discusses the features of the disease. A 24-year-old man presented with fever and a swollen 3-cm lymph node in the left side of the neck. The definitive diagnosis was established, after excision of level VB nodes on the left, by histologic examination. It revealed structurally intact tissue with scalloping in the paracortical area presenting activated T cells, focal monocytoid B cells, sinus histiocytosis and normally polarized follicles. Other two samples presented focal alterations of the tissue structure due to broad areas of necrosis, conspicuous nuclear debris, large lymphoid cells as well as numerous histiocytes. Immunohistochemistry revealed CD3+ and CD8+ T lymphocytes and histiocytes expressing CD68/PGM-1 (specific for macrophage lineage) and myeloperoxidase (MPO, specific for myeloid lineage). Correct, prompt diagnosis should be established through the findings of imaging and pathologic studies to avoid unnecessary investigation and ineffective therapies.
Kikuchi-Fujimoto disease (KFD) is a rare cause of lymphadenitis seen mostly in Asian populations (Kikuchi in Nippon
Kikuchi-Fujimoto disease (KFD) is a histiocytic necrotising lymphadenitis characterised by painful cervical lymphadenopathy, fever, malaise and weight loss. Infections, auto-immune pathogenesis and a genetic association have been implicated. A 12-year-old boy presented with a 1-month history of fever, abdominal pain, constipation and weight loss, and a painful lymph node was detected in the right axilla. Chest CT demonstrated multiple lymph nodes, especially in the left mediastinum. Salmonella enteritidis group D was detected in a blood culture and he was treated with ceftriaxone, followed by meropenem. An axillary lymph node biopsy demonstrated necrotising histiocytic lymphadenitis and KFD was diagnosed. He was discharged 35 days after admission. He was re-admitted 3 weeks later with recurrence of symptoms and headache and was found to have papilloedema of the left eye and auto-immune thyroiditis. Intravenous immunoglobulin (IVIG) 400 mg/kg/day was administered for 5 days. The fever and papilloedema slowly resolved and, subsequently, the thyroiditis, and he has remained well on follow-up. This is the first report of an association of S. enteritidis infection and papilloedema with KFD. IVIG may be required in prolonged or recurrent cases and in those with an auto-immune association.
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