|Year : 2015 | Volume
| Issue : 2 | Page : 150-152
Kikuchi-Fujimoto disease - A report of two cases
Department of General Surgery, Shri Sathya Sai Medical College and Research Institute, Ammapettai, Nellikuppam, Tamil Nadu, India
|Date of Web Publication||17-Jan-2016|
S-1, Block-4, Plot No. 76, Habitat Apartments, Sekaran Street, Krishnapuram, Ambattur, Chennai - 600 053, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Kikuchi-Fujimoto disease (KFD) or histolytic necrotizing lymphadenitis is a rare form of cervical lymphadenopathy that commonly affects young women. It is an idiopathic, self-limiting disease usually presenting with lymphadenitis, fever, and weight loss. I hereby report two cases of KFD, both of them presented with lymph node enlargements in the neck region. Excision biopsy was done after a trial of antibiotics, which gave a pathological diagnosis of KFD. KFD closely mimics, systemic lupus erythematosus and lymphoma, clinically and histologically. Although KFD is a benign disease, it has to be differentiated from these two diseases to avoid unnecessary chemotherapy, radiotherapy, and surgery.
Keywords: Cervical lymphadenopathy, histolytic necrotizing lymphadenitis, Kikuchi-Fujimoto disease
|How to cite this article:|
Kumar S. Kikuchi-Fujimoto disease - A report of two cases. Indian J Health Sci Biomed Res 2015;8:150-2
| Introduction|| |
Kikuchi's disease, also known as, apoptotic lymphadenitis, Kikuchi-Fujimoto disease (KFD), histolytic necrotizing lymphadenitis, previously called as subacute necrotizing lymphadenitis, is a rare, benign, and self-limiting condition of unknown etiology. It was first described by Kikuchi of Japan in 1972 and later the same year by Fujimoto et al. , It is commonly seen in young Asian women, although the disease has been reported throughout the world in all races.  A number of viral agents have been implicated as cause-including, cytomegalovirus (CMV), Epstein-Barr virus (EBV), Human herpes virus (HHV), varicella-zoster virus (VZV), para-influenza virus - but studies have failed to identify a specific pathogen.  There are reports to suggest a possible link between KFD and systemic lupus erythematosus (SLE).
| Case Reports|| |
A 35-year-old female presented with 4 weeks history of enlarged lymph nodes on both sides of the neck. She was otherwise asymptomatic. There was no head and neck pathology. She was clinically diagnosed as infective lymphadenitis and was prescribed 1-week course of antibiotics. After 10 days, she returned with persistent swelling also with no response to medications. After basic investigations (Hb, Bilgisayarlı Tomografi [BT], computed tomography [CT]), she was posted for lymph node excision biopsy, and two nodes from the left posterior triangle were excised and sent for histopathological examination (HPE), which revealed Kikuchi's disease ([Figure 1] low power view showing effaced architecture of fragmented lymph node with sheets of karryorrhectic debris). She was further investigated for SLE, tuberculosis, and Hodgkin's disease, all of which turned out to be negative. She was given anti-inflammatory drugs for occasional pain during immediate follow-up, and the lymph nodes regressed in 4 weeks.
|Figure 1: Low power view showing effaced architecture of fragmented lymph node with sheets of karryorrhectic debris|
Click here to view
A 20-year-old female nursing student presented with 4 weeks history of cervical lymphadenopathy on the right side. She has already completed two courses of different antibiotics prescribed by a physician, with no signs of improvement. She was otherwise asymptomatic and was posted for excision biopsy. The HPE report ([Figure 2] and [Figure 3] high power view of karryorrhectic debris with monocytoid lymphocytes, characteristic absence of neutrophils is diagnostic for KFD) revealed Kikuchi's disease and while investigating for other diseases, she was found to have weakly positive levels of anti-nuclear antibodies. Her lymph nodes regressed within 2 weeks of anti-inflammatory drugs, but, she continues to undergo regular follow-ups to look for recurrence of Kikuchi's disease and SLE.
|Figure 2: High power view of karryorrhectic debris with monocytoid lymphocytes, characteristic absence of neutrophils is diagnostic for Kikuchi-Fujimoto disease|
Click here to view
|Figure 3: High power view of karryorrhectic debris with monocytoid lymphocytes, characteristic absence of neutrophils is diagnostic for Kikuchi-Fujimoto disease|
Click here to view
| Discussion|| |
The most common presentation of KFD is with multiple, enlarged, tender lymph nodes in the neck, associated with fever and upper respiratory infection symptoms. Less common symptoms such as arthralgias, skin rashes, night sweats, malaise, weight loss, diarrhea, anorexia, chills, nausea, and vomiting have also been reported. Rarely, abdominal pain, chest pain, and hepatosplenomegaly are also seen. Cervical nodes are involved in 80% of cases (65-70% posterior triangle), axillary in 14%, and supraclavicular in 12% cases. 
The disease has an insidious onset like lymphoma, which mimics it both clinically and histopathologically. Other differential diagnoses are a metastatic carcinoma, infectious mononucleosis, toxoplasmosis, yersinia, tuberculosis, cat-scratch disease, AIDS, Kawasaki's disease, and Still's disease.
The etiology of KFD is not clear. Both autoimmune and infective mechanisms have been suggested. Although many viral agents (Yersinia enterocolitica, CMV, HHV, VZV, parainfluenza virus, and EBV) and parasites have been implicated, the pathogen remains controversial. Nevertheless, the frequency with which the association between KFD and SLE has been reported, suggests more than a chance occurrence. Hence, KFD patients should be followed long term for early detection of SLE, as is being done in our case-2.
The diagnosis of KFD is solely based on HPE of the excised lymph node. The role of fine needle aspiration cytology is limited, due to its low diagnostic accuracy rate, estimated at 56%.  Histologic findings consistent with KFD include (a) Paracortical necrosis, which may be patchy or confluent, the degree of necrosis varying considerably from one case to another, (b) histiocytes, crescent-shaped nuclei (crescentic nuclei), karyorrhexis, histiocytes, and macrophages containing phagocytozed debris from degenerated lymphocytes are seen, (c) other cells include lymphocytes, plasmacytoid monocytes, macrophages, and immunoblasts (predominantly T-cells). Another characteristic feature of KFD is the absence of neutrophils. Based on HPE, Kuo described three types of diseases - (1) Proliferative, (2) necrotizing and (3) xanthomatous.  Complete blood count, chest X-ray, ultrasonogram (USG) of abdomen, USG/CT neck, anti-nuclear antibodies, C-reactive protein, lactate dehydrogenase, anti-ds-DNA antibodies, etc., may be done to rule out other differential diagnoses.
KFD is a benign condition that usually resolves spontaneously within few months. Hence, only symptomatic and supportive treatment with nonsteroidal anti-inflammatory drugs is all that is needed to relieve pain and fever. Jang et al. suggested, using prednisolone in advanced cases to expedite resolution.  Corticosteroids and immunosuppressants have also been recommended in complicated cases.  Recurrence rates of 3-4% have been reported. 
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytosis. Nippon Ketsueki Gakki Zashi1 972;35:379-80.
Fujimoto Y, Kozima Y, Yamaguchi K. Cervical subacute necrotizing lymphadenitis: A new clinicopathological entity. Naika 1972;20:920-7.
Boone J, Charles SK. Kikuchi Disease. Mediscape Continually Updated Clinical Practice 2010.
Rao GS, Vohra D, Kuruvilla M. Is Kikuchi-Fujimoto disease a manifestation of systemic lupus erythematosus? Int J Dermatol 2006;45:454-6.
García CE, Girdhar-Gopal HV, Dorfman DM. Kikuchi-Fujimoto disease of the neck. Update. Ann Otol Rhinol Laryngol 1993;102:11-5.
Sousa Ade A, Soares JM, Sá Santos MH, Martins MP, Salles JM. Kikuchi-Fujimoto disease: Three case reports. Sao Paulo Med J 2010;128:232-5.
Kuo TT. Kikuchi′s disease (histiocytic necrotizing lymphadenitis). A clinicopathologic study of 79 cases with an analysis of histologic subtypes, immunohistology, and DNA ploidy. Am J Surg Pathol 1995;19:798-809.
Jang YJ, Park KH, Seok HJ. Management of Kikuchi′s disease using glucocorticoid. J Laryngol Otol 2000;114:709-11.
[Figure 1], [Figure 2], [Figure 3]