Kikuchi-Fujimoto disease (KFD) or Kikuchi histiocytic necrotizing lymphadenitis, is an extremely rare, benign and self-limiting disease which can be confused with lymphoma and sometime with infections such as tuberculosis. It has also been infrequently reported in association with autoimmune diseases. KFD shares sex and age predisposition as well as histological features with systemic lupus erythematosus. The co-existence of KFD with primary Sjögren's syndrome has only been rarely reported. Here we describe the case of a 33-year-old woman who was diagnosed with KFD and also found to have primary Sjögren's syndrome.
Learning points:
To understand the relationship between Kikuchi-Fujimoto disease (KFD) and Sjögren's syndrome.KFD shares some characteristics with autoimmune diseases.It is important to maintain a high index of suspicion of Sjögren's syndrome and diffuse lymphadenopathy in patients with KFD.