Familial Kikuchi-Fujimoto disease

Abstract

Kikuchi-Fujimoto disease (KFD) is a rare, self-limiting disease of unknown aetiology presenting with cervical lymphadenopathy, fever, vomiting, weight loss, night sweats and chills. Familial occurrence of KFD is reported very rarely in literature. We report two cases from the same family presenting with KFD. The two non-twin sisters presented with symptoms of fever, cervical lymphadenopathy, weight loss, nausea, vomiting, night sweats and chills 6 months apart. The elder sibling with KFD also manifested mononeuritis multiplex. Laboratory evaluation revealed raised erythrocyte sedimentation rate, C-reactive protein levels and leucopenia. Anti-nuclear antibody and anti-double stranded deoxyribonucleic acid antibody were negative. Histopathological findings were suggestive of Kikuchi -Fujimoto’s disease. The patients responded well to oral corticosteroid treatment.

Keywords

• Kikuchi-Fujimoto disease
• Necrotizing lymphadenitis
• Immunohistochemistry
• Familial