A catastrophic combination of Kikuchi–Fujimoto disease and systemic lupus erythematosus complicated with autoimmune hemolytic anemia in a pregnant woman

Abstract

Kikuchi–Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a benign, idiopathic, self-limiting, and systemic disorder involving lymph nodes (LNs) of unknown etiology. The first case was reported in Japan, with an increasing incidence globally. The disease is a challenge because of the nonspecific clinical features, and the disease is easily mistaken for other forms of lymphadenitis leading to misdiagnosis and mistreatment. It is associated with many autoimmune disorders, of which systemic lupus erythematosus (SLE) is the most common association. The diagnosis is based on the histological examination of LNs, which typically reveals necrosis surrounded by histiocytes with crescentic nucleus, immunoblasts and plasma cells, and absence of neutrophils. We report the case of a 24-year-old pregnant Indian female patient without any relevant past medical history to demonstrate the correlation between KFD and SLE complicated with autoimmune hemolytic anemia.