Kikuchi Fujimoto disease (KFD), otherwise called histiocytic necrotizing lymphadenitis is an uncommon, idiopathic disease affecting Japanese and east Asian individuals more commonly with a male to female ratio of 1:4.1-5.
Noninfectious causes include histiocytic necrotizing lymphadenitis which is a benign lymphadenitis, characterized by enlarged lymph nodes with histopathological findings of proliferation of lymphocytes and histiocytes, nuclear debris, and necrotic lesions affecting mainly young women ; systemic lupus erythematosus with lupus lymphadenitis can present with necrotic mediastinal lymph nodes .
In this study, the cytomorphological features observed in the cases were caseating epithelioid granulomas [47.6% (108/227)], granulomatous lymphadenitis [33.9% (77/227)], necrotizing lymphadenitis [1.8% (4/227)], and necrotizing suppurative lymphadenitis [16.7% (38/227)] of cases.
Treatment of histiocytic necrotizing lymphadenitis (Kikuchi's disease) with prolonged fever by a single course of methylprednisolone pulse therapy without maintenance therapy: experience with 13 cases.
Granulibacter bethesdensis is an emerging pathogen in patients with CGD that causes fever and necrotizing lymphadenitis. However, unlike typical CGD organisms, this organism can cause relapse after clinical quiescence.