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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 102-104

Kikuchi-fujimoto disease: A clinical enigma


1 Department of Pediatrics, Bhabha Atomic Research Centre Hospital, Mumbai, Maharashtra, India
2 Department of Pathology, Bhabha Atomic Research Centre Hospital, Mumbai, Maharashtra, India

Date of Submission04-Mar-2021
Date of Decision17-Apr-2021
Date of Acceptance04-May-2021
Date of Web Publication31-May-2021

Correspondence Address:
Dr. Sangeeta Priyadarshi Sawant
Department of Pediatrics, Bhabha Atomic Research Centre Hospital, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ipcares.ipcares_77_21

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  Abstract 

Background: Kikuchi Fujimoto disease (KFD) is a rare, benign self-limited disease characterized by prolonged regional lymphadenopathy associated with or without systemic signs or symptoms. It is a rare diagnosis in children. Due to the lack of pathognomonic clinical symptoms/signs, KFD poses a significant challenge to the clinician. Its diagnosis is confirmed by lymph node biopsy. Clinical Description: A 12.5-year boy presented with chronic cervical lymphadenopathy of 6-week duration, associated with mild-moderate fever, pain, and weight loss. He had raised erythrocyte sedimentation rate, leukopenia, lymphopenia, and thrombocytopenia. Mantoux test was positive. He was managed as a case of tuberculous lymphadenopathy till the lymph node biopsy confirmed the diagnosis of KFD. Management: The child recovered without medications. There has been no recurrence or relapse in 1.5 years of follow-up. Conclusion: This case report highlights the importance of considering the diagnosis of KFD in children presenting with persistent or chronic lymphadenopathy.

Keywords: Histiocytic necrotizing lymphadenitis, Kikuchi-Fujimoto disease, lymphadenopathy


How to cite this article:
Sawant SP, Amin AS, Gaddam PR, Chaturvedi UP. Kikuchi-fujimoto disease: A clinical enigma. Indian Pediatr Case Rep 2021;1:102-4

How to cite this URL:
Sawant SP, Amin AS, Gaddam PR, Chaturvedi UP. Kikuchi-fujimoto disease: A clinical enigma. Indian Pediatr Case Rep [serial online] 2021 [cited 2021 Sep 26];1:102-4. Available from: http://www.ipcares.org/text.asp?2021/1/2/102/317368

 Kikuchi-Fujimoto disease More Details (KFD) or histiocytic necrotizing lymphadenitis is a rare, idiopathic self-limiting disorder characterized by prolonged regional lymphadenopathy associated with/without systemic signs and symptoms. It was first described in 1972 by Kikuchi.[1],[2] Reported mostly in adult females below 40 years of age; it is a rare diagnosis in children and most studies in them are retrospective.[3],[4] Its cause is unknown; assumed to be an unidentified agent, probably a virus that triggers a self-limiting autoimmune process in a genetically predisposed person.[5]

There are no specific symptoms/signs, and this poses a challenge for clinical diagnosis and on many occasions; children are misdiagnosed or undergo extensive investigations. The diagnosis is established by histopathology of lymph node biopsy.

We report a case that highlights the importance of considering KFD in a pediatric patient presenting with chronic cervical lymphadenitis.


  Clinical Description Top


A 12.5-year-old boy presented with gradually increasing lymph node swelling on the right side of the neck associated with pain for 6 weeks. He had mild-moderate fever without chills/rigors and weight loss of 1.5 kg. There was no anorexia, lethargy, cough, rash, bleeding manifestations, joint pain, ear discharge, vomiting and loose stools. He had received antibiotics without any clinical response.

He weighed 52.4 kg with a height of 159 cm and body mass index of 20.78 kg/m2 (between 23 and 27 adult equivalent, Revised Indian Academy of Pediatrics Growth Charts). Vital parameters were normal. Neck examination revealed 2–3 right-sided cervical lymph nodes in the posterior triangle, largest measuring 3 cm × 3 cm, matted, firm with normal overlying skin. There was no hepatosplenomegaly and systemic examination was normal.

Management and Outcome

Investigations [Table 1] during this period of illness revealed mild anemia, a drop in total leukocyte count and lymphocyte count (lymphopenia). He also had thrombocytopenia in the initial week of illness. Peripheral smear demonstrated anisocytosis and hypochromasia, erythrocyte sedimentation rate (ESR), and serum aspartate aminotransferase (AST) were raised. Mantoux test was positive; 15 mm × 15 mm but chest X-ray was normal.
Table 1: Investigations of Kikuchi - Fujimoto disease in a 12.5-year-old boy

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Ultrasonography (USG) neck demonstrated variable-sized hypoechoic, noncalcific bilateral coalescent lymph nodes with areas of necrosis/breakdown. Mesenteric, suprapancreatic, retropancreatic, peripancreatic, and mesenteric lymph nodes were visualized on the USG abdomen. Lymph node aspiration cytology revealed reactive lymphoid cells, neutrophils with necrosis, and karyorrhectic debris.

Lymph node biopsy of the cervical lymph node was done. Histopathology of the excised lymph node [Figure 1] demonstrated effaced architecture with large areas of necrosis, abundant nuclear debris surrounded by abundant histiocytes and lymphocytes, and mixed inflammatory cells surrounding soft tissue. Granuloma, epithelioid cells, giant cell, and atypical cells were absent. The above findings were suggestive of KFD.
Figure 1: Lymph node showing areas of necrosis and abundant nuclear debris surrounded by lymphocytes and numerous histiocytes (×400)

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Ziehl–Neelsen stain for acid-fast bacilli and the Nucleic acid amplification (NAA) test i.e, Xpert MTB/RIF assay for  Mycobacterium tuberculosis Scientific Name Search TB) were negative on aspirate and biopsy material.

This child was empirically started on antitubercular medications based on the clinical history of fever, chronic lymphadenopathy, weight loss, and positive Mantoux test. He became afebrile within 48 h of biopsy and remained afebrile thereafter; hence, antitubercular medications were discontinued after the diagnosis of KFD was confirmed on histopathology.

Further investigations revealed raised C-reactive protein (CRP) and complement C3 and C4 levels. Anti-nuclear antibody (ANA) was negative.Culture for MTB using Flurometric Bactec Mycobacteria growth indicator tube (MGIT) liquid culture, did not reveal any growth at 3rd and 6th week.

He remained symptom free with no recurrence or appearance of new symptoms on follow-up for 18 months.


  Discussion Top


Our patient presented with chronic cervical lymphadenopathy associated with fever and was suspected to have tuberculosis initially. The diagnosis of KFD was subsequently established on histopathology of the excised lymph node. Cases having similar presentation have been reported previously, where diagnosis of KFD was established later on reviewing the biopsy report.[3],[6]

KFD, a rare condition in children, is assumed to be due to multifactorial etiology, i.e., hyperresponse of the immune system, induced by viral infections such as Epstein–Barr virus, Human Herpes virus 6 and 8, HIV, parvovirus, influenza virus, Human T-cell leukemia virus Type 1, Cytomegalovirus, Parainfluenza virus, and other organisms. It is predominantly a disease of people of Asian descent and Human Leucocyte Antigen class II genes namely HLA DPA*01 & HLA DPB1*0202, present frequently in them have been identified as related to the KFD.[5],[7]

Boys are affected twice compared to girls and have a mean age above 10 years. The presentation is mainly unilateral cervical lymphadenopathy which is firm, tender, matted, and varies in size between 0.5 and 4 cm. Axillary, abdominal, and inguinal lymph nodes may be involved. Associated symptoms such as fever, weight loss, fatigue, night sweats, nausea, vomiting, diarrhea, headache, arthralgia, myalgia, skin rash, and hepatosplenomegaly are observed.[3],[4]

KFD in both adults and children is associated with anemia, leukopenia, monocytosis, presence of atypical lymphocytes, elevated ESR, CRP, AST, alanine transaminase, and lactate dehydrogenase. Autoimmune antibody studies, including lupus erythematosus (LE) cell test and rheumatoid factor and ANA studies, are generally negative; these findings may help the clinician distinguish Kikuchi disease from systemic LE (SLE).[3],[4] The computed tomography scan findings of the neck include unilateral, homogeneous enlargement of multiple lymph nodes affecting levels II–V with perinodal infiltration, and nodal necrosis.[8]

The diagnosis of KFD is confirmed by excisional lymph node biopsy. Histopathological characteristics are specific and consist of areas of paracortical necrosis, abundant karyorrhectic nuclear remains, polymorphous cell population and fibrin deposits, apoptosis with destroyed cells, intense phagocytic activity, and neutrophils and plasmatic cells absence.[9]

The differential diagnosis of KFD includes infectious lymphadenitis of different etiologies, autoimmune lymphadenopathy (primarily SLE lymphadenopathy), and non-Hodgkin lymphoma. Histopathological features that distinguish KFD from lymphoma include incomplete architectural effacement with patent sinuses, the presence of numerous reactive histiocyte, relatively low mitotic rate, and the absence of Reed Stenberg cells. Special stains, immunohistochemistry, microbiological studies, and correlation with serologic and molecular studies can be helpful to confirm the diagnosis.[10]

Patients usually recover spontaneously without any sequelae. Fever usually decreases after the lymph node removal; the possible explanation being the removal of the focus which originates the inflammatory process. Thus, excisional biopsy has diagnostic as well as therapeutic benefits. In our patient, resolution of fever after biopsy was observed.

Few children require drugs such as nonsteroidal anti-inflammatory drugs, oral corticosteroids, hydroxychloroquine, or intravenous immunoglobulins depending on the severity.[4]

Recurrence has been reported in 7.5%–12.2% of children.[3],[4] Patients with recurrent episodes are more likely to have fever, fatigue, extranodal involvement, and remain symptomatic for a long duration. Sometimes, KFD may evolve into an autoimmune syndrome, mostly SLE which makes it necessary to follow-up with these children.[10]

Cervical lymphadenopathy is a common problem in the pediatric population. It is an important task in managing these cases; to identify and differentiate the benign conditions from the serious ones requiring specific treatment. KFD can be misdiagnosed as tubercular lymphadenitis in the regions where tuberculosis is prevalent[6] as was observed in our case. Early biopsy in the presence of persistent lymphadenopathy may be of great help to avoid unnecessary investigations in such situations.

Diagnosis of KFD is a challenge to the clinician. It should be considered in the differential diagnosis of chronic persistent lymphadenopathy in children.



Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytes: A clinico-pathological study. Nippon Ketsueki Gakkai Zasshi 1972;35:379-80.  Back to cited text no. 1
    
2.
Fujimoto Y, Kojima Y, Yamaguchi K. Cervical subacute necrotizing lymphadenitis. A new clinicopathological entity. Naika 1972;20:920-27.  Back to cited text no. 2
    
3.
Punnen A, Kanagalakshmi, Manipadam MT, et al. Clinico pathologic study of Kikuchi's disease in children in a tertiary hospital in South India. Int J Contemp Pediatr 2019;6:664-69.  Back to cited text no. 3
    
4.
Selvanathan SN, Suhumaran S, Sahu VK, et al. Kikuchi-Fujimoto disease in children. J Paediatr Child Health 2020;56:389-93.  Back to cited text no. 4
    
5.
Sopeña B, Rivera A, Vázquez-Triñanes C, et al. Autoimmune manifestations of Kikuchi disease. Semin Arthritis Rheum 2012;41:900-6.  Back to cited text no. 5
    
6.
Kaur S, Mahajan R, Jain NP, et al. Kikuchi's disease–A rare cause of lymphadenopathy and fever. J Assoc Physicians India 2014;62:54-7.  Back to cited text no. 6
    
7.
Lee KY, Yeon YH, Lee BC. Kikuchi-Fujimoto disease with prolonged fever in children. Pediatrics 2004;114:e752-6.  Back to cited text no. 7
    
8.
Kwon SY, Kim TK, Kim YS, et al. CT findings in Kikuchi disease: Analysis of 96 cases. AJNR Am J Neuroradiol 2004;25:1099-102.  Back to cited text no. 8
    
9.
Ojeda Lewis E, Vásquez Paz H. Kikuchi-Fujimoto disease: Pediatric case report. Medwave 2015;15:e6270.  Back to cited text no. 9
    
10.
Song JY, Lee J, Park DW, et al. Clinical outcome and predictive factors of recurrence among patients with Kikuchi's disease. Int J Infect Dis 2009;13:322-6.  Back to cited text no. 10
    


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