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 Table of Contents  
Year : 2018  |  Volume : 51  |  Issue : 1  |  Page : 26-28

Kimura's disease

Department of General Surgery, Jawaharlal Nehru Medical College, Wardha, Maharashtra, India

Date of Submission05-Apr-2017
Date of Decision13-May-2017
Date of Acceptance07-Jul-2017
Date of Web Publication09-Feb-2018

Correspondence Address:
Dr. Manish Swarnkar
Department of General Surgery, Jawaharlal Nehru Medical College, M4/10, JNMC Campus, Sawangi (Meghe), Wardha - 442 001, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/fjs.fjs_56_17

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Kimura's disease (KD) is a benign chronic inflammatory disorder attributed to an immune-mediated hypersensitivity which often presents as a tumor-like swelling in the head and neck region with or without lymphadenopathy, associated with hypereosinophilia and elevated serum immunoglobulin E. Most cases have been described predominantly in Chinese and Japanese people. KD has been confused with angiolymphoid hyperplasia with eosinophilia, from which it probably should be distinguished as a separate entity. The course is usually benign except for the potential cosmetic disfigurement. The diagnosis may be suggested by a fine-needle aspirate but is established by a biopsy. A case of KD in a 42-year-old male with recurrent left submandibular swelling is presented together with a brief review of the literature.

Keywords: Benign, chronic inflammatory, head and neck region, hypereosinophilia, recurrent

How to cite this article:
Swarnkar M, Agrawal A. Kimura's disease. Formos J Surg 2018;51:26-8

How to cite this URL:
Swarnkar M, Agrawal A. Kimura's disease. Formos J Surg [serial online] 2018 [cited 2022 Aug 8];51:26-8. Available from: https://www.e-fjs.org/text.asp?2018/51/1/26/225131

  Introduction Top

Kimura disease (KD) is an infrequent benign chronic inflammatory disease first reported by Kimm and Szeto in 1937 in China, but the disease was formally coined as “KD” when in 1948, a Japanese doctor named Kimura et al. published a systemic description of the disease.[1],[2] KD typically involves deep subcutaneous tissue and lymph nodes of the head and neck region with frequent regional lymphadenopathy and occasional salivary gland enlargement.[3]

Other localizations including the oral cavity, axilla, groin, limbs, and trunk have also been reported.[4] The disease is endemic in Asia (China, Japan) affecting most commonly men in third decade of life while sporadic in non-Asian population. Constitutional symptoms such as fever, night sweats, and weight loss are uncommon but often accompanied by elevated serum immunoglobulin E (IgE) levels and peripheral blood eosinophilia.[4] Our case was classical presentation with respect to the fact that it involved all three possible areas of preference: the subcutaneous tissue, salivary gland (submandibular gland), and lymph nodes.

  Case Report Top

A 40-year-old male patient came with a complaint of swelling in the left submandibular region [Figure 1] for 6 months which was firm in consistency, mobile, nontender, and nonadherent to the skin associated with cervical lymphadenopathy. There were no symptoms to suggest pulmonary tuberculosis such as chronic cough, night sweats, anorexia, or loss of weight. The patient had a history of similar swelling at same site 2 years ago, for which excisional biopsy was done. There was no axillary or inguinal lymphadenopathy or hepatosplenomegaly. Initial laboratory investigations revealed normal indices of full blood count with slightly raised eosinophils count and raised serum total IgE level (1750 IU/ml). Fine-needle aspirates were inconclusive, so surgical excision of the mass [Figure 2] was done under general anesthesia. Histopathological study of the specimen confirmed the diagnosis of KD by demonstrating lymphoid tissue with reactive follicular hyperplasia [Figure 3]. Within the germinal centers, deposits of abundant eosinophils and eosinophilc proteinaceous material were seen [Figure 3]. The interfollicular infiltrate was rich in eosinophils with admixed lymphocytes and eosinophilc microabscess [Figure 4].
Figure 1: Swelling in left submandibular region

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Figure 2: Removed specimen of mass along with submandibular gland

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Figure 3: Lymphoid follicle showing innumerable eosinophils with deposits of eosinophilic proteinaceous material (black arrows)

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Figure 4: Intense eosinophilic infiltration with formation of eosinophilic microabscesses (rectangles)

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  Discussion Top

The etiology and the pathogenesis of KD are unknown. The disease is classified as a benign reactive process. Allergic reactions, infections, and autoimmune reactions with an aberrant immune reaction have been suggested.[5] The findings of increased eosinophils, mast cells, and levels of interleukin-5 and IgE suggest an abnormal T-cell stimulation to a hypersensitivity-type reaction.[5] The histological picture with the formation of lymphoid follicles and intense aggregates of eosinophils (which sometimes form microabscesses), vascular proliferation, and fibrosis is highly suggestive of KD.[1] KD is endemic in the Far East, but only a few cases have been reported in nonorientals. For many years, it was thought that KD in orientals corresponded with the later stages of angiolymphoid hyperplasia with eosinophilia (ALHE) in Caucasians.[6] This may explain why the disease has not previously been reported as a separate entity in the English otolaryngological literature despite a predilection for the head and neck region. A path breaking study by Rosai et al.[7] eventually clarified this misconception, and thus KD and ALHE were established as two distinct entities. Diagnosis of KD is always a clinical dilemma with no specific diagnostic guideline. Clinically, among other differential diagnosis of KD would include Kikuchi disease, Mikulicz's disease, and most importantly Hodgkin and non-Hodgkin lymphoma. Fine-needle aspiration cytology (FNAC) is useful as an initial investigation of KD, with the main cytologic features of high number of eosinophils in a background of lymphoid cells. Nevertheless, histopathology examination of excised lesion is required for a definitive diagnosis as cytology may sometimes be difficult to interpret.

There is no agreement or uniform treatment protocol of KD. Therefore, its management involves a multidisciplinary approach. Treatment alternatives for KD consist of surgical resection, cytotoxic, and radiation therapy as well as regional or systemic steroids therapy.[8] In asymptomatic cases, conservative observation is often adequate as lesions occasionally undergo spontaneous resolution. The role of surgery is mainly for diagnostic and cosmetic purposes. Systemic corticosteroids may be used with or without other treatment modalities. Treatment with intralesional corticosteroid (triamcinolone acetonide) had been reported to have good outcome.[8] Relapses are common and recurrence rate is as high as 40% despite early treatment of the patients.[9] Although there is no consensus for the treatment of recurrent disease, the overall outcome is good as there is no association with malignancy.

  Conclusions Top

Even though rare, KD should be considered in the differential diagnosis of a recurrent head and neck subcutaneous mass. FNAC is a supportive tool, but histopathology is indispensable for diagnosis. The aim of management primarily is preserving function and cosmetics of the tissues affected. Although prognosis can be good, a complete cure is sometimes unattainable.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given his consent for his images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Kim HT, Szeto C. Eosinophilic hyperplastic lymphogranuloma, comparison with Mikulicz's disease. Proc Chin Med Soc 1937;1:329.  Back to cited text no. 1
Kimura T, Yoshimura S, Ishikawa E. On the unusual granulation combined with hyperplastic changes of lymphatic tissue. Trans Soc Pathol Jpn 1948;37:179-80.  Back to cited text no. 2
Meningaud JP, Pitak-Arnnop P, Fouret P, Bertrand JC. Kimura's disease of the parotid region: Report of 2 cases and review of the literature. J Oral Maxillofac Surg 2007;65:134-40.  Back to cited text no. 3
Abuel-Haija M, Hurford MT. Kimura disease. Arch Pathol Lab Med 2007;131:650-1.  Back to cited text no. 4
Rajpoot DK, Pahl M, Clark J. Nephrotic syndrome associated with Kimura disease. Pediatr Nephrol 2000;14:486-8.  Back to cited text no. 5
Nyrop M. Kimura's disease: Case report and brief review of the literature. J Laryngol Otol 1994;108:1005-7.  Back to cited text no. 6
Rosai J, Gold J, Landy R. The histiocytoid hemangiomas. A unifying concept embracing several previously described entities of skin, soft tissue, large vessels, bone, and heart. Hum Pathol 1979;10:707-30.  Back to cited text no. 7
Hashim HZ, Hoo FK, Lim SM, Mohamed MH, Ramachandran V, Ching SM, et al. Kimura disease – A case report and review of the literature. Pol Ann Med 2017;24:64-6.  Back to cited text no. 8
Göçmen H, Oguz H, Astarci M, Samim E. Kimura's disease: Case report and brief review of the literature. J Otolaryngol 2006;35:358-60.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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