1. ISSN 0975-8437 INTERNATIONAL JOURNAL OF DENTAL CLINICS 2011:3(1):115-116
CASE REPORT
Kimura’s Disease: A case report
Shilpa.C.Patel, Vishal.R. Dave
Abstract
Kimura’s disease is a rare inflammatory disorder of unknown cause, primarily seen in young
Asian males. The disease is characterized by a triad of painless subcutaneous masses in head and neck
region, blood and tissue eosinophilia and moderately elevated serum immunoglobulin E levels. Here, we
present a case report of this rare entity of unusual age presentation with classical histopathogical features.
Key Words: Kimura’s Disease; Eosinophilia; Parotid Gland; Lymphadenopathy
Received on: 22/09/2010 Accepted on: 06/12/2010
Introduction
Kimura’s disease (KD) is a rare chronic number. The histopathological feature of
angiolymphoid proliferative soft tissue disorder excisional biopsy was matched with that of
of unknown origin.(1) It occurs most often in incisional one. The patient was supplemented
young and middle aged asian male. It is most with oral Dexamethasone for total duration of 6
common in head and neck region, with a weeks (0.5 mg twice daily for 1 week and then
predilection for preauricular area. Typical tapered over 5 weeks). The patient was
clinical presentations are painless subcutaneous apparently normal without any local recurrence
masses, regional lymph node enlargement, blood & systemic involvement within 6 month of
and tissue hyper eosinophilia, and markedly follow-up period.
elevated serum IgE levels.(1) According to the Discussion
previous medical literatures, Kimura’s disease Kimura’s disease first described in1937
has a high recurrence rate so early & definitive in Chinese literature by H.T.Kimm and C.Szeto
diagnosis of the disease is vital for effective and they termed it as “eosinophilic hyperplastic
treatment plan.(1) Here we report a case of lymphogranuloma”.(2) The disease became
Kimura’s disease with clinico-pathological widely known as Kimura’s disease after Kimura
differential diagnosis. and colleagues reported two cases of unusual
Case Report granulation combined with hyperplastic changes
A 65 year old man reported to of lymphoid tissue.(1) The etiopathogenesis of
outpatient department with chief complaint of KD remains unknown and it is considered
painless swelling on right side of face in parotid nowadays as an allergic disease and it seems to
region since 4 months but it was painful on be a systemic immunological disorder.
mastication. Here we summarized details of Eosinophilia and increased serum IgE levels
present case in the following Table-1. With a make KD be considered a CD4 (+) T helper 2
provisional diagnosis of salivary gland (Th2) allergic reactions. Th2 cells would produce
neoplasm, an incisional biopsy was performed. interleukins (IL) IL-4, IL-5 and IL-13, which, in
The section stained with Haematoxylin & Eosin turn, would act in B cells favoring the production
revealed unencapsulated ill-defined lesion with of antigen-specific IgE. Th2 cell proliferation
lymphoid follicle formation, fibrosis and chronic and the overexpression of cytokines would play
inflammatory cell infiltration with predominant an essential role in the development of the
eosinophils (Figure 1a, 1b, 1c, 1d). Inter disease.(3)
follicular areas show increased number of It occurs predominantly as a unilateral
venules with endothelial cell proliferation. In manifestation in the head and neck and it is
some areas lymphoid follicle were infiltrated by frequently associated with regional
eosinophils with resultant eosinophilic micro lymphadenopathy with or without involvement
abscesses. With a diagnosis of Kimura’s disease, of salivary glands.(4) In the present case, e
the patient was undergone superficial lobe presence unilateral parotid gland swelling with
parotidectomy with removal of multiple regional regional lymphadenopathy led to the clinical
lymph nodes with vicinity. diagnostic dilemma of other pathologic entities
The excisional biopsy specimen affecting salivary glands & lymph nodes. The
received was firm nodular soft tissue measuring differential diagnosis of Kimuras disease
approximately 4.2X2X1.5 cm3 and two in frequently in previous literatures are
©INTERNATIONAL JOURNAL OF DENTAL CLINICS VOLUME 3 ISSUE 1 JANUARY-MARCH 2011 115

2. ISSN 0975-8437 INTERNATIONAL JOURNAL OF DENTAL CLINICS 2011:3(1):115-116
Lymphomas, salivary gland neoplasms, benign (AIL). Constant classical features of KD include
lymphoepithelial lesions (BLL / Mikulicz’s numerous lymphoid follicles, mixed
disease), Angiolymphoid hyperplasia with inflammatory infiltrate composed mainly of
eosinophilia (ALHE/ Epitheloid hemangioma) eosinophils and increased amount of post
and angioimmunoblastic lymphadenopathy capillary venules.(5)
Age/Sex location Physical findings lymphadenopathy Systemic involvement Laboratory investigation
65/Male Parotid (right) 3.5X3.5 cm firm, Regional No renal involvement Eosinophilia (36%) Elevated
nontender & normal lymphadenopathy erythrocyte sedimentation rate
overlying skin (ESR) Raised IgE levels
Table-1 clinical & investigation details of present case
Figure 1a. Lymphocytic infiltration with follicle formation and fibrosis .(4x), 1b. Well defined lymphoid follicle with
prominent germinal centre, (10x) 1c. Chronic Inflammatory cell infiltration with predominant eosinophils with
microabscesses.(40x), 1d. Interfollicular areas showing mixed inflammation with prominent eosinophils and
proliferation of venules.(40x), 1e. Interfollicular areas showing increased number of venules with endothelial cell
proliferation.(40x)
Till date there is no definite treatment Head, Department of Oral & Maxillofacial Pathology,
for KD is well established.(2) However the MGM Dental College & Hospital, Navi Mumbai, 2.
treatment plan should aim at preservation of vital Dr. Vishal.R.Dave, M.S, Assistant Professor,
structures associated with the lesions & cosmetic Department of ENT, GCS Medical College,
Ahmedabad, Gujarat, India.
rehabilitation, while preventing recurrences. References
Treatment options range from observation & 1. Chung YG, Jee WH, Kang YK, Jung CK, Park
follow-up of mild & symptomatic cases to GS, Lee A, Bahk WJ, Cho HM, Park JW.
conservative surgical approach, medication & Kimura’s disease involving a long bone. Skeletal
radiotherapy in symptomatic & recurrent cases. Radiology 2010; 39(5):495-500.
If the lesion is primary, localized or present in 2. Pitak-Arnnop P, Bellefqih S, Chaine A,
young age then surgical approach is more Dhanuthai K, Bertrand JC, Bertolus C. Head and
preferable. According to previous literature, only neck lesions of Kimura's disease: Exclusion of
surgical approach had high incidence of human herpesvirus-8 and Epstein-Barr virus by in
situ hybridisation and polymerase chain reaction.
recurrence.(1) If the lesion is recurrent with An immunohistochemical study. Journal of
systemic involvement, application of medication Cranio-Maxillofacial Surgery 2010; 38 (4):266-
like corticosteroid and immunosuppressive 70.
agents have been shown to decrease size of the 3. Briggs PL. Kimura disease is not angiolymphoid
lesion.(2) Irradiation should be considered in hyperplasia with eosinophilia: clinical and
patients resistant to the steroid or to prevent the pathological correlation with literature review
patient from deleterious effect of long term use and definition of diagnostic criteria. Anais
of steroid.(5) Brasileiros de Dermatologia 2006;81(2):167-73.
4. Kung ITM, Gibson J, Bannatyne P. Kimura's
Conclusion
Disease: A Clinico-Pathological Study Of 21
In conclusion this paper draws attention Cases And Its Distinction From Angiolymphoid
on such a rare chronic inflammatory disease Hyperplasia With Eosinophils.
which mimics neoplastic conditions. This disease Pathology1984;16(1):39-44.
should be considered in differential diagnosis of 5. Jani A, Coulson M. Kimura's disease. A typical
patients presented with head & neck mass and case of a rare disorder. Western Journal of
lymphadenopathy and investigated accordingly Medicine 1997; 166 (2):142-4.
as this disease has good prognosis. Knowledge Address for Correspondence
of signature features of Kimura’s disease put the Dr. Shilpa C Patel, M.D.S,
801, Osho Kabir, Plot No 42, Sector 10,
physicians in a better position to evaluate its
New Panvel, Maharashtra,India- 410206.
clinical outcome and optimal treatment regimen. Email: dr_shilpa0769@yahoo.com
Affiliations of Authors: 1. Dr. Shilpa.C.Patel,
M.D.S., Oral & Maxillofacial Pathology, Professor &
Source of Support: Nil, Conflict of Interest: None Declared
©INTERNATIONAL JOURNAL OF DENTAL CLINICS VOLUME 3 ISSUE 1 JANUARY-MARCH 2011 116