Kikuchi's disease associated with systemic lupus erythematosus and autoimmune-like hepatitis

The authors present a case of Kikuchi's disease associated with systemic lupus erythematosus (SLE) and autoimmune-like hepatitis. Kikuchi's disease, or histiocytic necrotizing lymphadenitis is occasionally associated with SLE and mildly elevated aminotransferases. A 17-year old woman presented with fever, arthritis and bilateral cervical adenopathy. Histopathological and immunohistochemical examinations of an excised lymph node showed evidence of Kikuchi disease. An elevation of hepatocytic enzymes (aminotransferases and gamma-glutamyl transpeptidase) associated with smooth muscle antibodies in a titer of 1/320 was present. Clinical symptoms and laboratory tests improved after "pulse" corticotherapy. The association described has not been described in the literature, but is considered possible due to the immune pathogenicity of the 3 simultaneous diseases. Kikuchi-Fujimoto's disease, or histiocytic necrotizing lymphadenitis (HNL), is a rare condition first described in 1972 independently by Kikuchi and Fujimoto. HNL is rarely associated with systemic lupus erythematosus (SLE). It is a benign illness characterized by fever and cervical adenopathy and has a self-limiting course.