Kikuchi-Fujimoto disease and systemic lupus erythematosus – an enigmatic association for clinicians and pathologists: Case report and short literature review

Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a benign condition of the lymphatic tissue whose cause has not been fully elucidated. Although it has been thought that various viral infections are involved in the onset of the disease, its immunological characteristics expressed by the proliferation of cytotoxic CD8-positive T lymphocytes and histiocytic phagocytosis place it at the border of autoimmunity. Sharing immunological mechanisms, clinical manifestations, and epidemiological data (both are described in young women) with systemic lupus erythematosus (SLE), the association of the two pathologies represents a challenge for both clinicians and pathologists.

We present the case of a young woman with SLE with immunological, haematological and articular involvement, who associates a few months after diagnosis the significant swelling of the submandibular glands and latero-cervical lymphadenopathy. Histopathological and immunohistochemical examinations subsequently certify the diagnosis of histiocytic necrotizing lymphadenitis associated with the autoimmune disease.