Kikuchi-Fujimoto's disease: report of familial occurrence in two human leucocyte antigen-identical non-twin sisters.

Abstract

Kikuchi-Fujimoto's disease (KFD) is a self-limiting condition usually presenting as cervical lymphadenopathy, with fever and leukopenia, in young adult females. No specific aetiology has been identified yet, although some authors believe that KFD is a 'forme fruste' of systemic lupus erythematosus. Certain human leucocyte antigen (HLA) types have also been associated with KFD. We herein report KFD in two non-twin sisters with HLA-identical phenotype, who presented 10 years apart. Neither patient had evidence of recent infection or connective tissue disease. The familial occurrence emphasizes the possibility of genetic predisposition and calls for a more extensive search for a specific cause of KFD.