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Table of Contents
CLINICAL RECORD
Year : 2003  |  Volume : 5  |  Issue : 1  |  Page : 35-37

“Kikuchi Fujimoto’s disease presenting as a benign neck mass”


1 Department of ENT, Buraidah Central Hospital - Buraidah A1 Qassim, KSA
2 Department of Surgery, Buraidah Central Hospital - Buraidah A1 Qassim, KSA
3 Department of Histopathology, Buraidah Central Hospital - Buraidah A1 Qassim, KSA

Date of Web Publication11-Jul-2020

Correspondence Address:
Fachartz Ibrahim Syed Akram
ENT Department P.O. Box 2290, King Fahad Specialist Hospital, Buraidah Al Qassim
KSA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1319-8491.289563

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  Abstract 


Kikuchi-Fujimoto’s Disease (KFD) is basically a benign, rare disorder of the lymph nodes of the cervical region. Otolaryngologists who are mainly involved in the diagnosis and management of neck masses are virtually unaware of this disease. We report a case of an Asian pregnant woman presenting with cervical lymphadenopathy associated with fever and sore throat. Aetiology, pathology, differential diagnosis, associated diseases and treatment options arediscussed in brief. This paper was written to increase the awareness of this disease among the practising otolaryngologists and to include this entity in the differential diagnosis of neck masses.

Keywords: Kikuchi-Fujimoto’s Disease, Pregnancy, Otolaryngologists, Neck masses, Differential Diagnosis


How to cite this article:
Akram IS, Asrar L, Raza SS, Ravichandran V, Faizuddin M. “Kikuchi Fujimoto’s disease presenting as a benign neck mass”. Saudi J Otorhinolaryngol Head Neck Surg 2003;5:35-7

How to cite this URL:
Akram IS, Asrar L, Raza SS, Ravichandran V, Faizuddin M. “Kikuchi Fujimoto’s disease presenting as a benign neck mass”. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 2003 [cited 2022 Dec 4];5:35-7. Available from: https://www.sjohns.org/text.asp?2003/5/1/35/289563




  Introduction : Top


Kikuchi Fujimoto’s Disease (KFD) or Histocytic necrotizing lymphadenitis is a rare, non-neoplastic, self-limiting disorder of unknown aetiology. [1] It usually presents as cervical lymphadenopathy, occasionally tender on palpation, associated with fever, sorethroat, myalgia and skin rash. It commonly affects young women between 30-40 years but children are not exempt. [2],[3] Although the disease occurs in all races worldwide it is more prevalent among Asians. The entity is well recognized by the pathologists but it is virtually unknown to the Otolaryngologists who are involved in the diagnosis and management of neck masses.[4],[5] As per literature to date KFD as such has no effect on pregnancy and vice versa but pregnancy has its own restrictions for investigation and treatment.[6],[7] Asian patient presenting with neck swelling when the primary investigation has failed to produce a positive diagnosis, KFD should be considered in the differential diagnosis. The diagnosis of KFD is difficult as there is no specific clinical feature or investigation for it. It rests solely on excision of the involved lymphnodes and accurate identification of the lesion by a pathologist. [1],[8],[9]


  Case Report: Top


A 28 year old Sri Lankan housewife was referred by a general practitioner to the ENT clinic, complaining of a two weeks history of slow-growing painful right-sided neck mass associated with fever, sore throat and bodyaches. There was no history of cough. She used to get fever up to 380C during night time. There was a history of amenorrhoea for 8 weeks.

On examination the patient was a young healthy female with a visible swelling in the right side of the neck. The swelling was 3cm X 2cm midway between theangle of right mandible and supraclavicular area in the anterior triangle. It was soft in consistency, tender and mobile; skin. There were some small nodes in the right upper jugulodiagastric region. ENT examination revealed congested pharyngeal wall. Posterior rhinoscopy and indirect laryngoscopy showed normal findings.

Laboratory data showed WBC 8.82x103 /ul with 56% neutrophils, 34.5% lymphocytes, 6.5% monocytes,2.4% eosinophils and 0.6% basophils. ESR was 63mm in the first hour. Throat swab was negative. Serological investigation for VDRL, Brucella, HIV Widal, Monospot, HbsAg, Toxoplasmosis were negative. Mantoux test was also negative. Radiological investigation was deferred due to her pregnancy in the first trimester.

In the meantime the patient was given a course of Amoxycillin 500mg TID with analgesics for one week. On follow up (one week later) her pharyngeal symptomsubsided but the neck gland remained unchanged. FNAC did not reveal any definitive findings. Further plan was to perform panendoscopy which the patient refused. General surgeon’s opinion was sought and she opted for an excisional biopsy under local anaesthesia. Patient gradually felt better after excision.

Clinically lymphoma / tuberculosis were suspected but to our surprise biopsy report showed a rare pathology “Kikuchi- Fujimoto’s disease”.

Biopsy specimen showed multiple small pale yellow areas in the cut surface of the node. Histopatholgy showed circumscribed pale areas of large histiocytic cells engulfing karyorrhectic debris with several apoptotic bodies between them. These areas of partial necrosis were surrounded by viable nodal parenchyma infiltrated by immunoblasts, plasmacytoid monocytes and usual small lymphocytes. There were no polymorphonuclear leucocytes. The patient recieved analgesics on PRN bases. One month and 3 monthfollow up in the ENT clinic were uneventful.
Figure 1: Figure shows pale irregular areas populated mainly by histocytes, alternating with dark compact areas of lymphocytic cells. 40 X

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  Discussion Top


In 1972 Kikuch & Fujimoto independently described a rare from of lymphadenopathy wich because of its characteristic histological appearance was termed “histiocytic necrotizing lymphadenitis”. Review of recent literature shows that though KFD is a disorder predominantly of young women of Asian origin, although cases among children have been reported.[2],[3] It occurs approximately four times in females as in males. KFD was once thought to be limited to the Asians but after 1982 it is known to occur worldwide. It presents with fever, sore throat, chills, myalgia and skin rash associated with unilateral cervical lymphadenopathy. Treatment of KFD is symptomatic and supportive. The disease has a self-limiting course, usually resolving within 1-3 months. Very rarely it may be associated with more extensive lymphadenopathy and hepatosplenomegaly. Recurrences and death due to the disease are exceedingly rare.[6],[l] Aetiology of this disease is unclear. Nonspecific hyperimmune reaction to infections, chemicals, physical and neoplastic agents have been proposed. Various organisms including HHV 6 & 8, parvovirus B 19 and EBV are also speculated to be the cause.[4],[7] Pahophysiologically a hyperimmune reaction causes the lymphnodes to become enlarged, inflamed and painful.
Figure 2: Figures shows a pale area populated by plasmacytoid monocytes and scattered immunoblasts. 200X

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Figure 3: Figure shows apoptosis and karyorrhectic debris. 200 X

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Recently Kikuchi-Fujimoto’s disease has been described as having 3 microscopic phases : 1.) The lymphoproliferative phase is characterized by predominance of lymphocytes, immunoblasts, histiocytes and debris but without coagulative necrosis. 2.) The necrotizing phase is characterized by coagulative necrosis. 3.)The xanthamatous phase has a predominance of foamy histiocytes. Usually the disease is diagnosed in 2nd or 3rd phase of the disease. First phase diagnosis is very difficult due to absence of characteristic features.[8],[9]

Conditions like SLE, hemophagocytic syndrome, Still’s disease are associated with KFD [6],[10], Very few cases of KFD arising during pregancy are reported [6],[7],

Pathogenesis of KFD is still unclear; apoptotic cell death seems to play a role in the pathogenesis of KFD. The necrosis associated with KFD is attributedto trans apoptotic death of the killer eel! target in the affected nodes. On this basis the name apoptotic lymphadenitis was also proposed for the disease.[11]

The case discussed here is a pregnant Asian female who presented to the ENT clinic with sore throat, fever, bodyaches associated with right-sided cervical lymphadenopathy. She was unresponsive to antibiotic therapy. Laboratory tests revealed normal WBC count with elevated ESR. Panel of serological tests were negative.

The mainstay of diagnosis of this rare disorder is histologic identificaton of the lesion. Excisional biopsy of the lymphnode helped us in arriving at a correct diagnosis. She was given symptomatic and supportive treatment after diagnosis. In one study steroids have been tried as effective option for early recovery. [12]

ENT surgeon plays an important role in diagnosing and managing neck masses. This rare entity is still not well-known to the otolaryngologists. The increasing number of cases seen worldwide should alert the ENT surgeon to keep this disease as one of the differential diagnosis of neck masses when the more common causes of cervical lymphadenopathy have been ruled out.

Particularly one should be aware of this disorder when dealing with patients of Asian origin presenting with cervical lymphadenopathy. Common diseases mimicking KFD like tuberculosis, malignant lymphoma, SLE, should be excluded before diagnosing this benign condition.[1],[3],[5],[10]

We conclude that KFD being a benign and rare disease, should be included in the differential diagnosis of neck masses, as the course and treatment differ greatly from those of tuberculosis, lymphoma and SLE. Otolaryngologists should be aware of this entity as the condition is usually self-limiting and associated with excellent long- term prognosis. Clinical features together with excision and histopathological examination of the lymphnode will lead to early diagnosis and obviate unnecessary investigations or therapeutic trials.

Acknowledgment:

We would like to thank Mrs. Reyminda R. Grageda, BSN-RN for typing this manuscript meticulously.



 
  References Top

1.
Dorfman RF, Berry GJ. Kikuchiis histiocytic necrotizing lymphadenaitis : An analysis of 108 cases with emphasis on differential diagnosis. Semin Diagn Pathol 1988;5: 329-45  Back to cited text no. 1
    
2.
O’Neill D, O’Grady J, Variend S. Child fatality associated with pathological features of histiocytic necrotizing lymphadenitis (Kikuchi- Fujimoto disease). Paed Pathol Lab Med 1998 ; 18(1): 79-88  Back to cited text no. 2
    
3.
A1 Salloom AA, Kichuchi’s Disease and Systemic Lupus Erythematosus in a Saudi child. Ann Saudi Med 1998; 18(1): 51-52.  Back to cited text no. 3
    
4.
Garcia CE, Girdhar-Gopal HV, Dorfman DM. Kikuchi-Fujimoto disease of the neck :Update Annal Oto Rhinol Laryngol 1993; 102:11-15.  Back to cited text no. 4
    
5.
Jayaraj SM, Loyd J, Frosh AC, Patel KS. Kikuchi-Fujimoto syndrome masquerading as tuberculosis. J Laryngol Otol 1999 ; 113 : 82-84.  Back to cited text no. 5
    
6.
Chmait RH, Meimin DL, Koo CN, Huffaker J. Hemophagocytic syndrome in pregnancy. Obstet Gynaecol 2000 95 (6Pt-2) 1022-4.  Back to cited text no. 6
    
7.
Al Nazar M A1 Haddad AM, A1 Althan SA, A1 Salem AH, A1 Faraj AA, A1 Saeed HH Kikuchi Fujimoto Disease Saudi Med J 2002; 23(4): 405-408.  Back to cited text no. 7
    
8.
Kuo T. Kikuchiis disease (Histiocytic necrotising lymphadenitis): a clincopathologic histologic study of 79 cases with analysis of histologic subtypes, immunohistolgy and DNA ploidy.Am J Surg Patho! 1995; 19: 798-809.  Back to cited text no. 8
    
9.
‘Menasce LP, Baneijee SS, Edmondson D, Harris M. Histiocytic necrotizing Lymphadentis (Kikuchi-Fujimoto Disease): continuing diagnostic difficulties. Histopatholgy 1998; 33(3): 248-54.  Back to cited text no. 9
    
10.
Abba AA, Afzal M, Alrnoharab F, Baez-Giangreco A. Kikuchiis disease : A clinicopathological analysis of 13 cases from Riyadh Central Hospital. Am Saudi Med J 1995; 3: 288-29.  Back to cited text no. 10
    
11.
Takakuwa T, Ohnuma S, Koike J, Hoshikawa M, Koizumi H, Involvment of cell mediated killing in apoptosis in histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease) Histopathology 1996 ; 28(1): 41-8.  Back to cited text no. 11
    
12.
Yong JJ, Kcun HP, Hyun JS Management of Kikuchi’s Disease using glucorticoid, J Lanygol Otol 2000; 114 : 709-11.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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