CASE REPORTS

Kikuchi’s Disease high grade, accompanied by headache and

non- productive cough. She was anorexic with
a weight loss of 3 kg. Her parents had noticed
Malathi Sathiyasekaran swellings on the left side of her neck, which
R. Varadharajan were painful and had gradually increased in
So. Shivbalan size over the last 2 weeks. There was no
history of vomiting, rash, arthralgia, breath-
lessness, mucosal bleed or contact with
An 11-year-old girl presented with high grade,
intermittent fever and cervical lymphadenopathy. She tuberculosis. On examination she was
had multiple enlarged left cervical lymphnodes. The conscious, febrile, not toxic. She had 5 to 7
examination of other systems was normal. Workup tender, enlarged left cervical lymphnodes of
for sepsis, malignancy and autoimmune disease were 2 × l cms size. There were no rashes or
negative. VA-IgM for EBV was positive and generalized lymphadenopathy. The exami-
histopathology of the lymph node was consistent with
nation of other systems was normal.
Kikuchi’s Disease.
Investigations revealed hemoglobin 11.2
Keywords: Kikuchi’s disease, Necrotising histiocytic
g/dL, TLC 2,700 cells/mm3, with a normal
lymphadenitis.
differential count and peripheral smear,
platelet count 3.5 lacs/mm3 and ESR 29 mm/
hr. Blood and urine cultures were sterile.
Kikuchi’s disease is a rare, benign Mantoux, Widal, Leptospirosis-IgM, HbsAg
clinicopathological condition presenting with and HIV by ELISA were negative. Chest
fever and lymphadenopathy. Its association skiagram was normal and ultrasonography of
with Systemic Lupus Erythematosus (SLE) the abdomen showed mild splenomegaly. Paul
makes it necessary to be aware of this Bunnel test, LE cell and ANA were negative.
condition and follow up these children. We Her complement levels (C3 and C4) were
report this child with Kikuchi’s disease. normal. Bone marrow study was normal and
Kikuchi’s disease should be considered as culture was sterile. VCA (Viral Capsid
differential diagnosis in young patients with Antigen) IgM for EBV (Epstein Barr virus)
cervical lymphadenopathy and fever of was positive 18 U/mL (>12U/mL = positive).
unknown origin. Excision biopsy of the enlarged left cervical
Case Report node was done and the histopathology
revealed lymphnode with essentially
An 11-year-old girl presented with history preserved architecture with multiple areas of
of fever for 45 days. Fever was intermittent, histiocytic aggregates showing necrosis and
karyorrhexis with few C-shaped monocytoid
From the Kanchi Kamakoti Childs Trust Hospital,
12-A, Nageswara Road, Nungambakkam, Chennai
cells. No granulomas were seen. There were
600 034, India. no neutrophils in the area of karyorrhexis.
Correspondence to: Dr. So. Shivbalan, F.49, First
Sheets of foamy histiocytes amidst blood
Main Road, Annanagar East, Chennai 600 102, vessels were seen. The findings were
Tamil Nadu, India. E-mail: shivbalan1@rediff consistent with necrotising histiocytic
mail.com lymphadenitis i.e., Kikuchi’s disease.
Manuscript received: June 3, 2003; Discussion
Initial review completed: July 30, 2003;
Revision accepted: August 22, 2003 Kikuchi and Fujimoto independently first

INDIAN PEDIATRICS 192 VOLUME 41__FEBRUARY 17, 2004

CASE REPORTS

reported Kikuchi-Fujimoto disease or play a role in the pathogenesis of Kikuchi’s

histiocytic necrotising lymphadenitis without disease via a cell mediated cytotoxic immune
granulocytic infiltration in Japan in 1972. It is response(8).
considered as a self-limiting benign systemic The important clinical features of
lymphadenitis, especially involving the Kikuchi’s disease are a female predominance
cervical nodes of unknown cause(1). Though (M:F 1:4) with a mean age of 30 years and
it has been reported worldwide, it still remains painless lymphadenopathy mostly in cervical
a poorly recognized clinicopathological entity region. The other reported features are fever,
and is confused with malignant lymphoma myalgia, sore throat, localized pain with mild
and systemic lupus erythematosus (SLE). leucopenia and increased LDH(1). Cutaneous
There are only few case reports of this disease lesions like facial rash, exudative erythema,
in children(2). erythematous papules, vasculitis, plaques and
nodules are observed in 40% of cases. They
The etiology of Kikuchi’s disease is not manifest simultaneously or after the illness(9).
known. Various etiological agents like human Arthralgia has also been reported. The
herpes virus (HHV6 and HHV8), herpes symptoms resolve spontaneously within two
simplex virus, adenovirus, parvovirus B19, to three months. Biopsy of the lymphnode is
cytomegalovirus, varicella zoster, dengue diagnostic. The pathological features of the
virus(3), bacteria such as Mycobacterium affected lymphnode are patchy or confluent
azulgai, yersinia and protozoa have been area of necrosis, varying amount of nuclear
linked to the disease(1). Kikuchi’s disease is debris in affected area, aggregates of
included in the protean manifestations of histiocytes, presence of medium - large sized
chronic EBV infection and could be a cause in transformed lymphocytes (immunoblasts) and
this child. Neoplastic conditions, autoimmune plasmacytoid T cells, absence of neutrophils
disorders and physiochemical agents have and eosinophils(8). Cytoplasmic tubuloreti-
also been postulated. These agents are thought cular structures resembling viral particles or
to stimulate a particular immune response aberrant organelle structures have been
resulting in this disease(1,4). Familial identified by electron microscopy(10). CT
occurrence has been reported and hence a scan of the affected lymphnode shows
genetic predisposition has been proposed(5). hypodense centers with peripheral ring
The inter relationship between SLE and enhancement corresponding to the central
Kikuchi’s disease, though strong is still necrosis. Kikuchi’s disease causes diagnostic
complex. Some authors feel that Kikuchi’s difficulties because of lack of specific signs,
disease is a forme fruste of SLE. There are also symptoms and serological markers. The
reports that suggest that Kikuchi’s disease diagnosis is based on histopathological
may progress to SLE, hence it is mandatory to findings, overlapping of histological features
exclude SLE and follow these children for requires differentiating it from a number of
several years to ensue early diagnosis of this infections, autoimmune and lymphoproli-
autoimmune disease, since the prognosis ferative disease. SLE is the most difficult
and management differ(5,6). Pathologically differential diagnosis, but the complete
malignant lymphoma resembles Kikuchi’s absence of neutrophils is a good clue, though
disease. Hemophagocytic syndrome is also this does not exclude the possibility of
hypothesised as a part of this diseas(7). It is evolution to SLE. In the majority of cases it is
postulated that the plasmacytoid monocytes a self-limiting disease, recurrence has been

INDIAN PEDIATRICS 193 VOLUME 41__FEBRUARY 17, 2004

CASE REPORTS

reported in 3% cases. There are reports of 4. Stephan JL, Jeannoel P, Chanoz J, Gentil-Perret
rapid resolution with steroids. The early A. Epstein-Barr virus-associated Kikuchi
disease in two children. J Pediatr Hematol
recognition of this disease helps in avoiding
Onco1 2001; 23: 240-243.
unnecessary investigations and treatment(4).
5. Amir AR, Amr SS, Sheikh SS. Kikuchi-
Contributors: MS and RV were involved in conception Fujimoto disease: report of familial occurrence
and design of the article, acquisition of data, analysis in two human leucocyte antigen- identical non-
and interpretation of data; SoS was involved in drafting twin sisters. J Intern Med 2002; 252: 79-83.
of the manuscript, critical revision of the manuscript
for important intellectual content and final approval 6. Abdullah A, Al Salloum. Kikuchi disease and
of the manuscript. systemic lupus erythematosus in a Saudi child.
Funding: None. Ann Saudi Med 1997; 181: 97-131.
Competing Interests: None stated. 7. Mahadeva U, Allport T, Bain B, Chan WK.
Hemophagocytic syndrome and histiocytic
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INDIAN PEDIATRICS 194 VOLUME 41__FEBRUARY 17, 2004