Kikuchi-Fujimoto disease presenting as pyrexia of unknown origin

Background: Kikuchi-Fujimoto disease, a benign self-limited lymphadenopathy is an uncommon cause of pyrexia of unknown origin (PUO). Methods: We retrospectively studied the case-records of 13 patients presenting with PUO who were diagnosed to have Kikuchi-Fujimoto disease on peripheral lymph node excision biopsy and report the salient clinical manifestations and histopathological findings in them. All of them received symptomatic treatment. Results: Their median age was 28 [interquartile range (IQR) 18.5-38.0] years. Women (11/13, 84.6%) were more frequently affected. All of them were human immunodeficiency virus (HIV) seronegative. Prior to presenting to us, two were being treated for lymph node tuberculosis with DOTS. Cervical lymph nodes were predominantly involved, the distribution being: right cervical (n=10, 76.9%); left cervical (n=4); and bilateral cervical (n=2). Axillary and generalized lymphadenopathy were rare being seen in 2 and 1 patient respectively. The median (IQR) erythrocyte sedimentation rate (n=11) was 53 (35-89) mm at the end of first hour. Salient histopathological features were paracortical patchy zones of eosinophilic fibrinoid necrosis with karyorrhectic debris, large numbers of histiocytes, including histiocytes with peripherally placed “crescentic” nuclei. Spontaneous regression of fever and lymphadenopathy was observed over a median (IQR) period of 8 (6.75-10.25) months in all of them. Conclusions: Kikuchi-Fujimoto disease is a rare but important cause of PUO presenting with peripheral lymphadenopathy. Women are most often affected and cervical lymph nodes are the most frequently involved site. Clinical suspicion and thoughtful collaboration between clinicians and pathologists are essential for accurate diagnosis, and to minimize unnecessary investigations and inappropriate aggressive treatment.