Histiocytic necrotising lymphadenitis (Kikuchi-Fujimoto disease) in Saudi Arabia: clinicopathology and immunohistochemistry

BACKGROUND: Kikuchi-Fujimoto disease (KFD) is a rare entity of uncertain cause that commonly presents as a benign self-limiting disease of unknown origin. The objective of this study was to document the clinical features, mode of presentation, histopathological and immunohistochemical (IHC) features of KFD at our institutions since little is known about this disease in our region. METHODS: We reviewed the histopathological reports of all lymph nodes resected at or referred to King Abdulaziz University Hospital between 1990 and 2003 and King Faisal Specialist Hospital & Research Center, Jeddah, Kingdom of Saudi Arabia between 2000 and 2003. All cases diagnosed as KFD were identified and the histological slides and clinical data were reviewed. IHC was performed for the proliferative marker Ki-67 and the apoptosis-related markers Bcl-2 and p53. RESULTS: In 2500 lymph node biopsies, 15 cases were diagnosed as KFD. The female to male ratio was 2.7:1. One patient presented with axillary lymphadenopathy and the others presented with cervical lymphadenopathy. Ages averaged 29 years and ranged from 13 to 46 years. There was no recurrence of the lymphadenopathy over 1 to 10 years of follow up. Bcl-2 and p53 were negative and Ki-67 was positive in 11 of 15 cases. CONCLUSION: The results support earlier findings that KFD is a self-limiting disorder that requires no specific management. We suggest a clinical follow-up for several years. The female predominance was striking. Apoptosis-regulating proteins are not helpful in the diagnosis. KFD usually expressed the proliferation-associated nuclear antigen Ki-67. Increased awareness of KFD will minimize the risk of confusing this entity with malignant lymphoma or other serious conditions.