We describe a young woman with two severe episodes of Kikuchi Fujimoto disease occurring 16 years apart. Both episodes were proven by biopsy, and on the second occasion the patient remained dependent on high-dose prednisone for more than 6 months in order to control inflammation and achieve a reduction in cervical lymph node size. The second lymph node biopsy showed leukocytoclastic vasculitis in addition to the typical features of Kikuchi Fujimoto disease, but, even though the clinical interpretation of this finding was unclear, we documented no clinical or laboratory evidence of the development of other serious systemic disease over 20 years of follow-up. Kikuchi Fujimoto disease is considered a disorder with a self-limited course and a favorable outcome. However, on the basis of our experience with this patient and data from peer-reviewed literature, we suggest that this generally accepted postulate should be revised.
Copyright 2003 Wiley-Liss, Inc.