Kikuchi-Fujimoto disease associated with Sjogren's syndrome: a case report and review of the literature.

Kikuchi-Fujimoto disease (KFD), known as subacute necrotizing histiocytic lymphadenitis, is an extremely rare, benign and self-limited disease, and has been infrequently reported with autoimmune diseases. Here we report a 17-year-old girl pathologically diagnosed as KFD who suffered recurrence of KFD and developed into Sjogren's syndrome (SS) after four years and then performed a systematic literature search about KFD associated with SS in which seven patients was reviewed in detail. The results show that SS may be prior to, simultaneous with or following KFD and it developed mainly in young (average age: 25 years), female patients (4/5) after KFD with an average latency of 43 months. Therefore, long follow-up and appropriate clinical and laboratory workup are highly encouraged to exclude underlying SS conditions in young women with KFD.