Background: The diagnosis of Kikuchi-Fujimoto disease (KFD) is challenging for a paediatrician. Recognizing unusual clinical presentations and features of KFD is essential for doctors to obtain a thorough understanding of this clinical entity.
Method: We reported a case recently diagnosed in our ward with manifestation of recurrent thrombocytopenia and Mobitz type II atrioventricular block, which is very unusual in childhood KFD. We also used three powerful Chinese Journal Search Engines (Cqvip.com, Wanfang.data and ScienceChina) to search the cases reported in China from 1989 to 2006.
Results: One hundred seventy-three patients younger than 16 years old with pathologic proof of KFD were identified. Among them, 138 cases with detailed clinical records from eight different provinces and cities were analyzed, and of which 97 patients were followed-up for average of 10 years and the outcomes are discussed.
Conclusions: KFD is characterized by a spectrum of distinctive features, but also a variety of systemic manifestations. A hyperimmune reaction to unidentified agents as well as an autoimmune component may play an important role in this disease. Early corticosteroid therapy may improve the long-term prognosis of KFD in children.