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Kimura Disease of the Parotid and Retroauricular Region

Article in Archives of Otolaryngology - Head and Neck Surgery · February 2007

DOI: 10.1001/archotol.133.1.86 · Source: PubMed

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Demet Yazici Melek Ergin

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 CLINICAL NOTE

Kimura Disease of the Parotid and

Retroauricular Region
A Case Report
Demet Yazici, MD; Ulku Tuncer, MD; Melek Ergin, MD

K
imura disease is a rare, slowly progressive, immune-mediated disorder of unknown
origin, usually affecting young Asian male patients. The typical triad of this disorder
comprises (1) painless unilateral cervical adenopathy or subcutaneous masses pre-
dominantly in the head and neck region; (2) blood and tissue eosinophilia; and (3)
marked elevated serum IgE levels.1 Because this disease can mimic a neoplastic process, early di-
agnosis can save the patient from any harmful and unnecessary invasive diagnostic procedures.
We describe herein a Turkish man with Kimura disease who developed nephrotic syndrome and
was treated with steroid therapy.

REPORT OF A CASE then, and 1 year later he developed ne-

phrotic syndrome and received steroid
A 25-year-old Turkish man was admitted treatment for 1 month. After the cessa-
to our clinic with painless, nodular swell- tion of steroid therapy, the mass reap-
ing that covered the left periauricular and peared, and the patient was referred to our
parotid regions (Figure 1). The swell- clinic.
ing began to appear 10 years prior to ad- His vital signs, blood pressure, and find-
mittance, and he developed nephrotic syn- ings from general physical examination
drome 1 year after the swelling began. He were all normal. A nodular, painless, non-
did not have any history of pain, fever, fluctuant lump beginning at the left ret-
night sweats, or weight loss, and his ap- roauricular region and extending to the left
petite was good. He was born in Turkey; parotid region was palpable in the head
both of his parents were of Turkish ori- and neck region. There was no lymphade-
gin; and he did not have any history of trav- nopathy elsewhere, and the overlying skin
eling abroad. of the mass was normal. The hemoglobin
An incisional biopsy specimen had been level of the patient was 15.3 g/dL; plate-
taken from the swollen area 10 years be- let count was 225⫻109/L; and white blood
fore admittance, at another clinic, and the cell count was 9.6 ⫻ 109/L. Differential
histopathologic findings were reported at analysis showed 49.9% eosinophils, 28.8%
that time as “non-specific chronic lymph- segmented neutrophils, 17.6% lympho-
adenitis.” The patient was thought to have cytes, and 3.2% monocytes. The erythro-
tuberculosis and was treated with antitu- cyte sedimentation rate was 2 mm/h. The
berculosis drugs. The mass diminished IgE level was markedly increased at 1.4
then but swelled again 1 year later. An- mg/dL (normal, ⬍0.06 mg/dL). Results of
other incisional biopsy specimen was urinalysis were within normal limits. Se-
taken, but the reported histopathologic rum electrolyte and albumin levels were
findings were the same again. He under- normal, as were results of liver and renal
went antibiotic therapy then, also, but we function tests. The titers for Toxoplasma
could not find the records for the drug regi- gondii and Epstein-Barr virus were non-
men administered. The mass diminished reactive. The C-reactive protein level
was normal. There were no abnormal
Author Affiliations: Departments of Otolaryngology (Drs Yazici and Tuncer) and findings in the sputum, urine, or stool cul-
Pathology (Dr Ergin), Cukurova University, Adana, Turkey. ture. The chest radiograph and the skel-

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 etal survey did not show any patho-
logic findings. The computed
tomographic (CT) scan of the neck
showed a heterogeneous enhanc-
ing mass, measuring 9 ⫻ 2 cm,
covering the left parotid region and
the left retroauricular region
(Figure 2). The mass could not be
differentiated from the masseter
muscle. The chest CT image of the
patient was normal.
Fine-needle aspiration biopsy
findings were nonspecific, so an in-
cisional biopsy specimen was taken
under local anesthesia from the
nodular lesions of the retroauricu-
lar region. The pathologists were
unable to provide a diagnosis from
this specimen, so another biopsy
specimen was taken under general
anesthesia. The histopathologic
evaluation of this specimen showed
follicular hyperplasia and eosino- Figure 1. The retroauricular lump of the patient before steroid therapy.
philic infiltration with fibrotic
hyalin background (Figure 3), and
a diagnosis of Kimura disease
was made.
As a first step, steroid therapy was
prescribed for the patient (oral pred-
nisolone, starting from 1 mg/kg per
day and tapering off one fourth of
the dose every 4 days until the dose
reached 2 mg/d). The lump dimin-
ished during prednisolone therapy
(Figure 4), so the surgical exci-
sion of the mass was cancelled. The
patient still uses low-dose steroid
therapy (2 mg/d), and he has been
free of disease for 6 months.

COMMENT

Kimura disease was first described

by Kim and Szeto2 in the Chinese
medical literature as “eosinophilic Figure 2. Computed tomographic scan demonstrating a large soft tissue lesion in the parotid and
hyperplastic lymphogranuloma” in retroauricular regions.
1937. However, it was not recog-
nized worldwide until Kimura et al3 the head and neck region and also eosinophilia, allergic granulomato-
described similar cases under the involves subcutaneous tissues; peri- sis of Churg-Strauss, Mikulicz dis-
title “On the Unusual Granulation auricular, inguinal, and axillary ease, and follicular lymphoma.1,10 For
Combined With Hyperplasic lymph nodes; parotid and subman- many years, there had been an over-
Changes of Lymphatic Tissue [in dibular glands; and, rarely, sites such lapping between Kimura disease
Japanese]” in the Japanese medical as the auricula, scalp, pharynx, epi- and angiolymphoid hyperplasia with
literature in 1948. glottis, and groin.7-9 eosinophilia, but today these 2 dis-
Kimura disease is mostly seen In the differential diagnosis, one eases are considered separate enti-
among young men of Asian origin, must consider diseases such as ties with distinctive clinical and his-
with the male-female ratio ranging Hodgkin lymphoma, tuberculosis, tologic features.9
from 3.5:1.0 to 9.0:1.0.4,5 Some cases angioimmunoblastic T-cell lym- Histopathologically, in Kimura
have also been reported among phoma, Langerhans cell histiocyto- disease, normal tissue architecture
white, black, Hispanic, and Arab pa- sis, Castleman disease, histiocytosis is usually preserved; however, fol-
tients.6 This disease usually affects X, angiolymphoid hyperplasia with licular hypertrophy of lymphatic tis-

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 ing the production of cytokines and
causing eosinophil activation and an
IgE response). However, the real
cause is still unknown.12
To diagnose this disease, histo-
pathologic evaluation is necessary.
Cytologic evaluation by fine-
needle aspiration biopsy can be tried,
but because fine-needle aspiration
produces a specimen of such small
mass, an incisional or excisional bi-
opsy specimen must be taken from
the lesion so that the diagnosis may
be confirmed histopathologically.6
The ultrasonographic features
of this disease do not show any
characteristic properties. On CT
images, nodal and extranodal mani-
festations of Kimura disease are at-
tenuated soft tissue lesions with vari-
able contrast enhancement, and
heterogeneous gadolinium enhance-
Figure 3. Follicular hyperplasia with prominent germinal centers (hematoxylin-eosin, original ment can also be seen. The mag-
magnification ⫻200).
netic resonance findings of Kimura
disease are variable and depend on
the fibrosis and vascularity of the
mass.13
The treatment options for Kimura
disease are intralesional or sys-
temic steroid therapy, surgical re-
section of the mass, cryotherapy, ra-
diotherapy, chemotherapy, and
antihistaminic drugs such as cetiri-
zine. Steroid therapy for Kimura dis-
ease can shrink the mass, but recur-
rence is common when the drug
treatment is stopped.11,14 The mass
gets smaller with radiotherapy, but
this treatment is not recommended
in young patients. Cyclosporin A and
vincristine can also be used in pa-
tients with Kimura disease.15
In conclusion, Kimura disease is
a benign disease, and there are no re-
ports of malignant transformation.
The cause of this disease is unclear.
Figure 4. The mass diminished after the patient began steroid therapy. An incisional biopsy specimen from
the mass must be taken for patholo-
sue with infiltration of lympho- mal-change disease; IgM, IgA, and gists to provide the exact diagno-
cytes, histiocytes, and large numbers IgE nephropathy; and mesangiopro- sis. There are many therapeutic
of eosinophils is typical, and the liferative glomerulonephritis may options for this disease, but we rec-
germinal center of lymph nodes may also develop in patients with Kimura ommend the nonsurgical ones,
be necrotic with central eosino- disease. Renal disease may pre- especially oral steroid therapy, as the
philic abscess.6 cede, follow, or coincide with this first-line therapies.
Renal disease may also be en- disease.5,11
countered in patients with Kimura The cause of this disorder is still Submitted for Publication: March
disease. Proteinuria has been re- unclear. There are speculations that 5, 2006; final revision received July
ported in 12% to 16% of the it might have an allergic (parasite, 13, 2006; accepted August 7, 2006.
patients, and 60% to 78% have virus, fungus, or toxin) or autoim- Correspondence: Demet Yazici, MD,
developed nephrotic syndrome.5,10 mune origin, and there are articles Tarsus Devlet Hastanesi, KBB
Different renal abnormalities such as describing this disease as a disor- Klinigi, Tarsus, Mersin 33400, Tur-
membranous nephropathy; mini- der of type 2 T-helper cells (affect- key (demetyazici@[Link]).

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 Author Contributions: Dr Yazici had 2. Kim HT, Szeto C. Eosinophilic hyperplastic ease with salivary gland involvement. Br J Surg.
lymphogranuloma, comparison with 1981;68:495-497.
full access to all the data in the study Mikulicz’s disease. Chin Med J (Engl). 1937; 9. Gumbs MA, Pai NB, Saraiya RJ, Rubinstein J,
and takes responsibility for the in- 23:699-700. Vythilingam L, Choi YJ. Kimura’s disease: a case
tegrity of the data and the accuracy 3. Kimura T, Yoshimura S, Ishikwa E. On the un- report and literature review. J Surg Oncol. 1999;
of the data analysis. Study concept and usual granulation combined with hyperplastic 70:190-193.
design: Yazici, Tuncer, and Ergin. Ac- changes of lymphatic tissues [in Japanese]. Trans 10. Matsuda O, Makiguchi K, Ishibashi K, et al. Long-
Soc Pathol Jpn. 1948;37:179-180. term effects of steroid treatment on nephrotic syn-
quisition of data: Yazici. Analysis and 4. Li TJ, Chen XM, Wang SZ, Fan MW, Semba I, Kitano drome associated with Kimura’s disease and a re-
interpretation of data: Yazici. Draft- M. Kimura’s disease: a clinicopathologic study of view of the literature. Clin Nephrol. 1992;37:
ing of the manuscript: Yazici. Criti- 54 Chinese patients. Oral Surg Oral Med Oral Pathol 119-123.
cal revision of the manuscript for im- Oral Radiol Endod. 1996;82:549-555. 11. Lu HJ, Tsai JD, Sheu JC, et al. Kimura disease in
5. Yamada A, Mitsuhashi K, Miyakawa Y, et al. a patient with renal allograft failure secondary to
portant intellectual content: Yazici, Membranous glomerulonephritis associated chronic rejection. Pediatr Nephrol. 2003;18:
Tuncer, and Ergin. Statistical analy- with eosinophilic lymphfolliculosis of the skin 1069-1072.
sis: Tuncer and Ergin. Administra- (Kimura’s disease): report of a case and review 12. Jani A, Coulson M. Kimura’s disease: a typical
tive, technical, and material support: of the literature. Clin Nephrol. 1982;18: case of a rare disorder. West J Med. 1997;166:
Yazici. 211-215. 142-144.
6. Chen H, Thompson LD, Aguilera NS, Abbon- 13. Hiwatashi A, Hasuo K, Shiina T, et al. Kimura’s dis-
Financial Disclosure: None re- danzo SL. Kimura disease: a clinicopathologic ease with bilateral auricular masses. AJNR Am J
ported. study of 21 cases. Am J Surg Pathol. 2004; Neuroradiol. 1999;20:1976-1978.
28:505-513. 14. Ben-Chetrit E, Amir G, Shalit M. Cetirizine: an ef-
7. Kuo TT, Shih LY, Chan HL. Kimura’s disease: in- fective agent in Kimura’s disease. Arthritis Rheum.
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