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Kikuchi-Fujimoto disease
Disease definition
Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disorder, characterized by regional cervical lymphadenopathy with tenderness, usually accompanied with mild fever and night sweats. Less frequent symptoms include weight loss, nausea, vomiting, sore throat.
ORPHA:50918
Classification level: DisorderSummary
Epidemiology
Kikuchi-Fujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence among Japanese and other Asiatic individuals. Prevalence is unknown. Only isolated cases are reported in Europe.
Etiology
The clinical, histopathological and immunohistochemical features appear to point to a viral etiology, a hypothesis that still has not been proven.
Diagnostic methods
KFD is generally diagnosed on the basis of an excisional biopsy of affected lymph nodes. Its recognition is crucial especially because this disease can be mistaken for systemic lupus erythematosus, malignant lymphoma or even, though rarely, for adenocarcinoma. Clinicians' and pathologists' awareness of this disorder may help prevent misdiagnosis and inappropriate treatment. The diagnosis of KFD merits active consideration in any nodal biopsy showing fragmentation, necrosis and karyorrhexis, especially in young individuals presenting with posterior cervical lymphadenopathy.
Management and treatment
Treatment is symptomatic (analgesics-antipyretics, non-steroidal anti-inflammatory drugs and, rarely, corticosteroids). Patients with Kikuchi-Fujimoto disease should be followed-up for several years to survey the possibility of the development of systemic lupus erythematosus.
Prognosis
Spontaneous recovery occurs in 1 to 4 months.
A summary on this disease is available in Deutsch (2006) Español (2006) Français (2006) Italiano (2006) Nederlands (2006)
Detailed information
Guidelines
- Anesthesia guidelines
- Czech (2016) - Orphananesthesia
- English (2016) - Orphananesthesia
Disease review articles
- Review article
- English (2006) - Orphanet J Rare Dis
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