Kikuchi-Fujimoto Disease (Histiocytic Necrotizing
Lymphadenitis). Case Study and a Literature Review
Olejárová M.1, Campr V.2, Pavelka K.1
1Revmatologický ústav, Praha, ředitel prof. MUDr. K. Pavelka, DrSc. 2Ústav patologie a molekulární medicíny 2. lékařské fakulty UK, Praha, přednosta prof. MUDr. R. Kodet, CSc. |
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Summary:
Kikuchi-Fujimoto disease is a rare feverish disease characterised by lymphadenopathy, the most frequently cervical, exanthem, arthralgias and arthritis. It affects especially young women. Pati-ents háve high erythrocyte sedimentation rate (ES) and leucopenia, antibodies are missing. Cour-se of the disease is usually very benign and can subside spontaneously. However, clinical picture is usually very dramatic and can suggest infectious, autoimmune or malign systemic disease; also association with some of autoimmune diseases was described. Iťs occurrence is sporadic in all the world, the most of cases were seen in Asia, in the Czech Republic it has not been yet described. Therapy consists in antibiotics administration followed with corticoid therapy and usually can restore patients to perfect health. However, exacerbations háve also been described. We describe a čase of a 60 year old man, a past top sportsman, who has nevěr been seriously ill except sport traumas and prosthesis implantation for coxarthritis reasons. The last two years he has suffered from exanthem and leucopenia of an unclear origin. In May 2002 he become feverish and arthritis, lymphadenopathy, splenomegalia and exanthem progression, high ES rate and high sérum level of C-reactive protein (CRP) appeared in him. His condition was first evaluated as septic condition (founded staphylococci in two blood cultures), however, cause of potential sepsis has hot been identified. The patient was treated with antibiotics with improvement of his total health condition after second treatment regiment. A neck node biopsy was doně because of suspicion on lym-phoprolipherative disease and histiocytic necrotizing lymphadenitis of Kikuchi type was found. Autoantibodies assessment was completely negative. After antibiotic and corticoid therapies his clinical condition quite quickly standardized and ES rate and sérum CPR level decreased. 4 month after lowering the dose of prednisolon a temporary exacerbation of the disease appeared and again disappeared after increasing the dose of corticoid.
Key words:
Kikuchi-Fujimoto disease - Histiocytic necrotizing lymphadenitis
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