Kikuchi-Fujimoto Disease is a rare benign, condition of necrotising histiocytic lymphadenitis. A case of a 55 year old gentleman is described here. He presented with fevers, weight loss and tender cervical lymph nodes. Kikuchi-Fujimoto disease was diagnosed after cervical lymph node biopsy. Symptomatic treatment was provided and an uneventful full recovery was made. Of significant note, this patient's daughter had Kikuchi-Fujimoto disease almost a decade earlier. Although Kikuchi-Fujimoto disease has been reported widely, we believe this is the first familial report in the literature of a case of Kikuchi-Fujimoto disease occurring in a parent and child

A Santana

F Altuntas

G Famularo

HC Lin

M Kikuchi

Mojisola Akinsola

N Louis

Sonna Ifeacho

Theingi Aung

TT Kuo

Y Fujimoto

Y Kucukardali

English

PubMed

Springer - Publisher Connector

BioMed CentralCases Journal
ssOpen AcceCase Report
Kikuchi-Fujimoto Disease: A case report and review of the 
literature
Sonna Ifeacho*, Theingi Aung and Mojisola Akinsola
Address: Department of General Medicine, Heatherwood Hospital, London Road, Ascot, SL5 8AA, UK
Email: Sonna Ifeacho* - sonna@doctors.org.uk; Theingi Aung - t.aung@hwptr.nhs.uk; Mojisola Akinsola - m.akinsola@hwptr.nhs.uk
* Corresponding author    
Abstract
Kikuchi-Fujimoto Disease is a rare benign, condition of necrotising histiocytic lymphadenitis. A case
of a 55 year old gentleman is described here. He presented with fevers, weight loss and tender
cervical lymph nodes. Kikuchi-Fujimoto disease was diagnosed after cervical lymph node biopsy.
Symptomatic treatment was provided and an uneventful full recovery was made. Of significant note,
this patient's daughter had Kikuchi-Fujimoto disease almost a decade earlier. Although Kikuchi-
Fujimoto disease has been reported widely, we believe this is the first familial report in the
literature of a case of Kikuchi-Fujimoto disease occurring in a parent and child.
Background
The presenting complaint of neck masses in association
with non-specific systemic signs and symptoms prompt
investigation towards the more common diagnoses. How-
ever, rarer conditions must still be considered especially
when a patient's condition fails to abate. Here in we dis-
cuss a case of male patient who presented with a neck
mass that was not attributable to the more common
causes.
Case presentation
A 55 year old gentleman presented with a two week his-
tory of fevers, anorexia and weight loss. There were no
other complaints. He had been previously fit and well and
was on no medication. On examination he was lethargic,
but otherwise looked well. He was afebrile and haemody-
namically stable. Significant findings were generalised
lymphadenopathy, palpable in the occipital, posterior
auricular, cervical, axillary and inguinal regions. There
was 2 cm smooth, tender hepatomegaly. Blood tests
revealed neutropenia, hyponatraemia, raised alkaline
phosphatase and C-reactive protein. Electrocardiogram
and chest radiographs were normal. His initial manage-
ment consisted of fluid restriction and regular paraceta-
mol, whilst results of further tests were awaited. These
included blood and sputum cultures, autoimmune and
viral screen.
Subsequently the axillary and inguinal lymphadenopathy
resolved along with reduction in size and tenderness of
the cervical lymph nodes. There were persistent intermit-
tent temperature spikes and a two day episode of self-
resolving diarrhoea.
Blood, urine and stool cultures were negative. Sputum
cultures grew respiratory tract flora and were negative for
acid-fast bacilli. Paul Bunnell test was negative. The
autoimmune screen was negative, as was toxoplasma and
cytomegalovirus screens.
Computerised tomography demonstrated generalised
lymphadenopathy in the supraclavicular area; axillae,
Published: 26 September 2008
Cases Journal 2008, 1:187 doi:10.1186/1757-1626-1-187
Received: 2 June 2008
Accepted: 26 September 2008
This article is available from: http://www.casesjournal.com/content/1/1/187
© 2008 Ifeacho et al; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 3
(page number not for citation purposes)
Cases Journal 2008, 1:187 http://www.casesjournal.com/content/1/1/187posterior mediastinum, para-aortic areas and deep in the
pelvis and groin. The abdominal viscera were normal.
Excisional biopsy of a cervical lymph node confirmed a
diagnosis of Kikuchi-Fujimoto disease (KFD). Histologi-
cal analysis showed histiocytic granulomatous infiltration
with widespread necrosis of the lymph node extending
beyond its capsule into the surrounding fat. No active
treatment was instigated and the patient was discharged
home after a two week in-patient stay.
At follow-up he reports no symptoms, remains well and
there are no abnormalities on clinical examination.
The patient's daughter had KFD seven years earlier and
was a patient in the same hospital. She presented with ten-
der cervical lymphadenopathy of two months duration,
associated with two stone weight loss, fevers and night
sweats. She had no past medical history of note.
The only finding on examination was left posterior auric-
ular lymphadenopathy. Routine blood tests, blood cul-
tures and an autoimmune screen were negative. Blood
tests showed a mild leucopenia. ESR and CRP were mildly
raised. She had normal renal and liver function.
A lymph node biopsy taken from the posterior auricular
area established a tissue diagnosis of KFD. She improved
during her in patient stay, with her bloods returning to
normal prior to discharge. At review one week and one
month later she had made a full recovery.
Discussion
KFD is a benign histiocytic necrotising lymphadenitis.
KFD is rare, but most common in Asia. In the early 1970s
both Kikuchi and Fujimoto first described cases of KFD in
Japan [1,2]. Its aetiology has not yet been fully deter-
mined, however it is believed it may be of viral origin,
EBV, HHV6 and 8 have been suggested. Raw fish was pos-
tulated as a cause, but the recent literature doesn't support
this [3]. An autoimmune aetiology is also likely as it has
been reported in association with SLE. It tends to affect a
young population under 30 years of age, including chil-
dren, although the latter are less commonly affected.
There are reported cases in an older age group and preg-
nant women too [4]. Early reports suggest affected female
cases are more common; however more recently this view
has changed to one of equal prevalence in both genders.
The most common signs and symptoms are lymphaden-
opathy, fever, sweats, malaise, anorexia, weight loss,
hepatomegaly and leucopenia [3]. The viral aetiology of
KFD is supported by its non-specific self-resolving symp-
toms, which are of slow, insidious onset.
A definite diagnosis is made by tissue biopsy, indeed
whole lymph node biopsy. Histopathological assessment
of affected lymph nodes reveals characteristic findings.
There are three main patterns identified, proliferative,
necrotizing and xanthomatous. The proliferative picture is
seen in approximately a third of cases and has a dominant
inflammatory infiltrate. Half of cases show necrotizing
pattern and the xanthomatous type is rare and has abun-
dant foam cells [5]. Immunoblast cell changes seen in
lymph nodes mimic those of malignancy and are a source
of diagnostic confusion. Cellular protein structures have
been noted in the cytoplasm of lymphocytes and histio-
cytes that have also been found in those cells of patients
with SLE. This adds strength to the hypothesis that KFD is
a self-limiting SLE-like disorder.
As the symptoms are non-specific and some of the histo-
logical features are similar to other diseases it is easy to
misdiagnose KFD with SLE or lymphoma. This is impor-
tant as the treatment of KFD is symptomatic and support-
ive, spontaneous recovery is usual, while the latter two
conditions require prompt specific treatments.
Long term follow-up of these patients is necessary as
recurrent cases of KFD have been reported and there is
some belief that KFD may be a precursor for SLE, as both
diseases have had concurrent and co-existing disease pat-
terns in the same patients [6-8]. In a review of KFD cases
by Kucukardali et al the reported overall mortality rate
associated with KFD is 2.1% [9].
Conclusion
KFD is uncommon, but should feature in a list of differen-
tial diagnoses of tender lymphadenopathy, especially
affecting the cervical region. Its treatment differs signifi-
cantly from the other conditions that would be on that list
such as SLE, lymphoma and TB. Lymph node biopsy will
aid accurate diagnosis, but if confusion with SLE occurs
differentiation can be made with the aid of blood tests for
complement levels amongst others.
Recurrence has been reported. KFD has been reported
within a family, but not between parent and child. This
case is believed to be the first reported one. It may suggest
a genetic predisposition or a common environmental fac-
tor. Long term follow up will give us more data.
Learning points
• KFD is an important diagnosis to make as treatment is
conservative and this differs to the management of other
conditions it may be confused with.
• KFD requires a tissue diagnosis and therefore lymph
node biopsy should be considered in patients with cervi-
cal lymphadenopathy and systemic upset.Page 2 of 3
(page number not for citation purposes)
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• When clinical features suggest a diagnosis of KFD, a fam-
ily history should be sought.
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
All authors read and approved the final manuscript.
Consent
Written informed consent was obtained from the patient
for publication of this case report and any accompanying
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.
References
1. Kikuchi M: Lymphadenitis showing focal reticulum cell hyper-
plasia with nuclear debris and phagocytosis.  Nippon Ketsueki
Gakkai Zasshi 1972, 35:378-380.
2. Fujimoto Y, Kozima Y, Yamaguchi K: Cervical subacute necrotiz-
ing lymphadenitis. A new clinicopathological agent.  Naika
1972, 20:920-927.
3. Louis N, Hanley M, Davidson NM: Kikuchi-Fujimoto disease: a
report of two cases and an overview.  J Laryngol Otol 1994,
108:1001-1004.
4. Altuntas F, Sari , Canoz O, Yildiz O, Eser B, Cetin M, Unal A:
Kikuchi-Fujimoto disease: a rare but important cause of
fever and lymphadenopathy in pregnant women.  Am J Haema-
tol 2006, 81(2):118-120.
5. Kuo TT: Kikuchi's disease (histiocytic necrotizing lymphaden-
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histiolytic subtypes, immunohistology and DNA ploidy.  Am J
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6. Santana A, Lessa B, Galrao L, Lima I, Santiago M: Kikuchi-Fujim-
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7. Famularo G, Giustiniani MC, Marasco A, Minisola G, Nicotra GC, De
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(page number not for citation purposes)


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