KIKUCHI-FUJIMOTO DISEASE (KFD): A Rare Case Report

Authors: Dr. Gunja Jain; Dr. Mayank Gupta; Dr. Laxmikant Goyal; Dr. Jai Purohit; Dr. Sudhir Mehta
DIN
IMJH-JUL-2017-5
Abstract

Kikuchi-Fujimoto disease (KFD) is a rare disease. It has a worldwide distribution with a higher prevalence in Asians. KFD is usually a self limiting disease and benign in nature. Clinically it presents as regional cervical lymphadenopathy and sometimes may presents as generalized lymphadenopathy as well. Night sweats and low grade fever may also be associated in some cases. A case of a 36 year old female had attended in SMS Hospital. She presented with fever, weight loss and tender cervical lymph nodes. Diagnostic significance of KFD lies in that it may mimic some of the common diagnosis like SLE (Systemic Lupus Erythematosis), Tuberculosis, lymphoma and rarely adenocarcinoma. Diagnosis is usually made by biopsy of lymph node which shows fragmentation, necrosis and karyorrhexis. On thorough investigation this case was found of Kikuchi-Fujimoto disease (KFD) which is a rare disease. So case presenting with fever, weight loss and tender cervical lymph nodes should also be investigated for Kikuchi-Fujimoto disease (KFD).

Keywords
Kikuchi-Fujimoto disease (KFD).
Introduction

Cervical lymphadenopathy is a common manifestation of varied etiology. The illness ranges from benign infectious etiology to malignant lymphoma. 

KFD is also known as histiocytic necrotizing lymphadenitis. It was first described by Kikuchi and Fujimoto in 1972 so called Kikuchi-Fujimoto disease (KFD). It is a rare, benign and self limited disorder of unknown cause. 

It is characterized by tender cervical lymphadenopathy, constitutional symptoms such as fever and night sweats1 . Patient of KFD is usually younger with mean age of 21 years2 . It has a slight female preponderance3 . It is a self limiting disease with no specific therapy. 

It may be an under-diagnosed condition and as it has an excellent prognosis. It should be differentiated from malignant lymphomas. Its knowledge should be there in both clinicians as well as pathologists who will help in preventing misdiagnosis and inappropriate treatment1 . Recurrence is around 4%. It should always be considered in differential diagnosis of patients with younger age, cervical lymphadenopathy and biopsy features of necrosis, fragmentation and karyorrhexis4, 5, 6 .

Conclusion

KFD is uncommon, but should feature in the list of differential diagnosis of tender cervical lymphadenopathy as its treatment is conservative and differs significantly from the other conditions that would be on that list such as TB, lymphoma, metastasis and SLE. Lymph node biopsy will aid in accurate diagnosis of necrotizing histiocytic lymphadenitis or Kikuchi-Fujimoto Disease.

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