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 Table of Contents  
Year : 2020  |  Volume : 15  |  Issue : 4  |  Page : 347-353

Kimura disease: A rare presentation in the rheumatology clinic

1 Department of Internal Medicine, Command Hospital, (SC), Pune, Maharashtra, India
2 Department of Rheumatology, Command Hospital, (SC), Pune, Maharashtra, India
3 Department of Otorhinolaryngology, Command Hospital, (SC), Pune, Maharashtra, India
4 Department of Pathology, Command Hospital, (SC), Pune, Maharashtra, India
5 Department of Radiology, Armed Forces Medical College, Pune, Maharashtra, India

Date of Submission21-May-2020
Date of Acceptance18-Jul-2020
Date of Web Publication18-Dec-2020

Correspondence Address:
Dr. Arun Hegde
Department of Rheumatology, Command Hospital, (SC), Pune - 410040, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_132_20

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Kimura disease (KD) is a rare chronic inflammatory disorder of unknown cause, primarily seen in young Asian males. The disease is characterized by painless subcutaneous swelling in head and neck region, accompanied by regional lymphadenopathy and frequent salivary gland enlargement. Blood and tissue eosinophilia, and elevated immunoglobulin E (IgE) levels, are common associations. Characteristic histopathological findings of biopsy specimens obtained from the subcutaneous swellings or lymph nodes include eosinophilic infiltrates, follicular hyperplasia, and proliferation of postcapillary venules. The course is usually waxing and waning, albeit benign. Early diagnosis may spare the patient from unnecessary invasive procedures. We herein, describe a case of KD in an 18 years old male, who presented with subcutaneous swelling in the both cheek (right more than left) in 2014, underwent multiple surgical interventions for the same, before being reassessed and finally diagnosed as KD in 2020, based upon peripheral blood eosinophilia, raised serum IgE levels and histopathological findings. He subsequently made a good recovery on oral steroids.

Keywords: Angiolymphoid hyperplasia with eosinophilia, eosinophilia, immunoglobulin E, Kimura disease, subcutaneous cheek swelling

How to cite this article:
Kumar M, Hegde A, Dwivedi G, Sengupta P, Bhanu KU. Kimura disease: A rare presentation in the rheumatology clinic. Indian J Rheumatol 2020;15:347-53

How to cite this URL:
Kumar M, Hegde A, Dwivedi G, Sengupta P, Bhanu KU. Kimura disease: A rare presentation in the rheumatology clinic. Indian J Rheumatol [serial online] 2020 [cited 2022 Jun 29];15:347-53. Available from:

  Introduction Top

Kimura disease (KD) is a rare chronic inflammatory disorder of unknown etiology, that was first reported by Kim 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 systematic review of the disease.[1],[2] KD has been seldom reported from India, and since its first histopathological diagnosis, only 200 cases having been reported worldwide in literature.[3] It is usually seen in young adults, with most patients in age group of 20–40 years, although children might also be affected. Men are affected more commonly than women, with a 3:1 ratio.[4],[5] The disease is endemic in Asians but occurs sporadically in other racial groups. KD typically involves deep subcutaneous tissue of the head and neck region, along with regional lymphadenopathy and occasional involvement of extracutaneous sites.[3] Peripheral blood eosinophilia and elevated immunoglobulin E (IgE) levels are constant features of KD. Systemic involvement in the form of renal disease is common with an incidence of about 20%.[6],[7] The diagnosis of KD is often difficult, and the biopsy or excision of the involved mass or lymph node for a histopathological examination (HPE) is necessary.[8] It is commonly confused with an entity referred to as angiolymphoid hyperplasia with eosinophilia (ALHE), due to overlapping clinical features and similar histopathological (HPE) findings.

In our case too, despite the lesion having progressively involved the subcutaneous tissue of the cheek, parotid gland, and cervical lymph nodes, the diagnosis was initially misconstrued as a case of ALHE for 6 years, before being revised to KD.

A 19-year-old male, presented for the first time in 2014 to the oral surgeon, with complaints of a subcutaneous swelling in right cheek region of 1-year duration that was insidious at onset and gradually progressive in nature. A mass lesion measuring 4.3 cm × 2 cm × 2.7 cm involving right cheek, deep tissues of right cheek without involvement of skin or mucosa was revealed on ultrasound (USG) scan. There were discrete enlarged bilateral submandibular and jugular nodes, without necrosis or calcification. (Majority were 0.8–1.2 cm in size). The same findings were confirmed on contrast-enhanced computed tomography (CECT) face. Fine needle aspiration cytology (FNAC) from cheek swelling was suggestive of soft tissue lesion, likely fibroblastic in origin. He underwent excision per orally in 2014 and the patient remained asymptomatic. No systemic therapy was offered.

The patient again presented with similar complaints, this time, in form of diffuse swelling on left side of cheek in 2018, to the otorhinolaryngologist. Clinical examination revealed a well circumscribed 5 cm × 4 cm firm swelling on left cheek inferior to zygoma, 3 cm lateral to ala of the nose, 3 cm anterior to angle of mandible and 2 cm superior to ramus of mandible, which was non tender and nonfluctuant [Figure 1]. He had no history of any constitutional symptoms. FNAC of lesion was suggestive of benign salivary lesion, (Warthins tumor). Magnetic resonance imaging (MRI) (face and neck) revealed a 2.6 cm × 3.3 cm × 4.6 cm, well defined lesion in subcutaneous tissue plane of Buccal area anterior to masseter muscle with extension into Stensons duct and anterior aspect of superficial pole of parotid gland. On Right side, similar lesion measuring 1.7 cm × 1.6 cm × 3.5 cm was seen in buccal area superficial to masseter muscle without any cervical lymphadenopathy. Differential diagnoses considered by the radiologist were of minor salivary gland tumor and accessory parotid swelling. He underwent trans-oral excision of the left cheek swelling [Figure 2]. A repeat HPE revealed a lesion with follicular hyperplasia, proliferation of vascular channels lined by plump endothelial cell and accompanying severe inflammatory infiltrate comprising of eosinophils predominantly, consistent with a diagnosis of ALHE [Figure 3] and [Figure 4]. He subsequently remained symptom free.
Figure 1: Swelling in left cheek involving Buccal region, extending to parotid and upper jugular lymph nodes

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Figure 2: Removed specimen of mass from left cheek swelling

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Figure 3: Photomicrograph of biopsy from cheek showing a lymphoid follicle surrounded by numerous eosinophils (H and E, × 100)

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Figure 4: Photomicrograph of biopsy from cheek showing sheets of eosinophils and few capillary sized vessels lined by plump endothelial cells (H and E, ×400)

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In 2019, he again presented to the otorhinolaryngologist, with a 5 cm x 5 cm globular swelling in right cheek in submucosal plane involving right buccal region, extending to parotid region and right upper jugular lymph nodes [Figure 5]. MRI (face and neck) was again suggestive of recurrent/residual lesion involving both cheeks, in a known case of ALHE (postoperative) [Figure 6]a and [Figure 6]b. FNAC (right level II lymph node) showed polymorphous population of lymphoid cells composed of centroblasts, centrocytes, mature lymphoid cells and immunoblasts, along with clusters of histiocytes, with no atypical lymphoid cells, granulomas or giant cell. FNAC of Right parotid swelling showed cellular with polymorphous population of lymphoid cells composed of centroblasts, centrocytes, mature lymph cells, and few entrapped clusters of epithelial cells. Based on previous history the recurrent nature of the swelling in the head and neck region causing cosmetic disfigurement, and substantiated by the HPE findings a diagnosis of KD was entertained (a close mimic of ALHE). The patient was evaluated for serum IgE levels and absolute eosinophil count (AEC). AEC was 2900/cu mm. Peripheral blood smear was suggestive of eosinophilia. His hematological examination revealed hemoglobin-14.2 g/l, total leukocyte count -8000/cumm (neutrophils, 33.6%; lymphocytes, 21.2%; monocytes, 7.7%; and eosinophils, 36.9%). Acute phase reactants showed C reactive protein of 1.5 mg/l (Normal: 1.00–10.0 mg/l). Biochemical examination revealed normal renal and liver functions, urine routine and microscopy were normal, with no proteinuria. IgE levels were elevated-1267.7 IU/ml (normal value: 1.5–3.78 IU/ml) with normal serum IgG, M and A. Serum IgG4 levels were also normal. The HPE was also reviewed, and the diagnosis was revised to KD in view of prominent lymphoid follicles, rich eosinophilic infiltrate and numerous proliferating capillaries lined by plump endothelial cells in the background of raised serum IGE levels.
Figure 5: Swelling in right cheek involving buccal region, extending to parotid and upper jugular lymph nodes

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Figure 6: (a) T2-weighted image Coronal image and (b) T2-weighted image axial image of face shows heterogeneous signal of the soft tissue over right cheek

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Based on the above, the patient was referred to the Rheumatology OPD, where he was finally diagnosed as a case of KD. He was managed with oral steroids in the form of Tab prednisolone at a dose of 35 mg with regression in the size of the lump over the left cheek, over the next 3 months [Figure 7]. Repeat IgE levels at 3 months were 438.7 IU/L. Repeat MRI showed absence of any significant residual mass over cheek [Figure 8]a and [Figure 8]b. Presently, he is on tapering doses of steroids.
Figure 7: Post systemic steroid therapy

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Figure 8: (a) Posttreatment T2-weighted image coronal image of face shows minimal residual lesion, (b) T2-weighted image axial image shows significant reduction in the size of the mass over the right cheek

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  Discussion Top

KD is a benign, male predominant, chronic inflammatory soft tissue disorder of unknown origin, occurring in second to fourth decades of life, that usually presents with cosmetically disfiguring, painless, subcutaneous nodules in the region of the head and neck accompanied by frequent regional lymphadenopathy and extracutaneous sites of involvement like parotids, oral cavity, nasal sinuses and orbit. The most commonly involved lymph nodes are the epitrochlear, axillary and inguinal node.[8],[9] Renal involvement in form of minimal change disease and membranous nephropathy can be there, that manifests as nephrotic syndrome, in 12%–16% of the cases.[7] The clinical course is usually benign. Biopsy of subcutaneous mass or enlarged lymph nodes reveal characteristic features such as florid germinal center hyperplasia and eosinophilic microabscesses, IgE in germinal centers, and possible vascular component in the form of hyperplastic small blood vessels lined by endothelial cells.[10],[11],[12],[13] Other diagnostic clues include raised serum eosinophil counts and elevated serum IgE levels.[6] The exact etiology and pathogenesis of KD is still not clear, possible theories being that it could be of allergic or autoimmune origin [Figure 9]. The possible theory is that of a CD4 type 2 T Helper cell proliferation, with resultant cytokine overproduction (IL4, IL5, RANTES, tumor necrosis factor alpha), resulting in production of lymphoid follicles and high IgE.[9],[14] KD may closely mimic neoplasms, and early diagnosis could spare the patient from needless multiple interventions. USG, computed tomography, and MRI might be diagnostic and can help in staging the extent and progression of the disease, as well as the lymph node involvement.[3]
Figure 9: Representation of possible pathogenesis: Persistent exposure to an unknown allergen could trigger the occurrence of Kimura's disease (KD). The predominant Th2 immune response mediates the release of IL-4, IL-5, and IL-13, as a result, elevated IgE and abundant eosinophils with mast cells cause a type 1 hypersensitivity reaction. This could be the initial immune response in KD. Th2 cells also produce IL-5 on which eosinophilic activity is dependent.

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Our patient was a young male with no complaints apart from that of cosmetic disfigurement. He had no systemic symptoms, history of insect bite, previous allergies, or any offending drug intake. His renal functions were normal. Clinically, our case had both lymph node (Right level II lymph node) and extraneous (Right parotid swelling) involvement, with HPE showing eosinophilic infiltrates and intact germinal centers, with no atypical cells. The other differential diagnosis that merit consideration with these presenting clinical features and histopathological findings include classical Hodgkin lymphoma, eosinophilic granulomatosis with polyangiitis, IgG4 related disease, Castlemann's disease and parasitic infestations like Toxoplasma gondii lymphadenitis. Absence of ReedSternberg cells excluded Hodgkin's disease and absence of giant cells, granulomas, necrotic foci, and organization of inflammatory component in a florid pattern of lymphoid follicles ruled out other differential diagnosis.[10],[15],[16],[17] Serum IgG4 levels were normal and that coupled with the absence of a lymphoplasmacytic infiltrate excluded IgG4 related disease. The differentiating features are discussed in detail in [Table 1]. The various other differential diagnoses with their salient clinical features are discussed in [Table 2]. KD has often been confused with ALHE, so it is important to know the differences in between the two.[14],[18] The same have been highlighted in [Table 3].
Table 1: Clinical and pathological differences between Kimura disease and IgG4-related disease

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Table 2: Clinical features and pathological findings of other differential diagnosis

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Table 3: Clinical and pathological differences between Kimura disease and Angiolymphoid hyperplasia with eosinophilia

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There is no standardized treatment protocol of Kimura's disease, and its management involves a multidisciplinary approach. Surgical management is mainly for cosmetic and diagnostic purpose. Systemic corticosteroids have been indicated in relapsing cases, but risk of relapse when steroid treatment is withdrawn has been observed. When both surgical and medical management fail, local radiation has been postulated to be effective.[19] Our case underwent excision of the right cheek swelling in 2014 and left cheek swelling in 2018, with recurrence of swelling over right cheek in 2019. This time he was referred to the rheumatologist, and was started on systemic corticosteroid therapy with good response in the form of reduction in cheek swelling and reduction in IgE levels.

  Conclusion Top

Although rare, KD should be considered in the differential diagnosis of a recurrent head and neck subcutaneous swelling. Being an orphan disease, the rheumatologist should be aware of this clinical entity, as it seldom presents to the rheumatology outpatient department. Elevated serum IgE levels and tissue eosinophilia are important adjuncts in arriving at a diagnosis, in addition to the classical HPE findings. A multi-speciality approach, involving close cooperation between the rheumatologist, otorhinolaryngologist, radiologist, oral surgeon and pathologist, along with a high index of suspicion, is required to arrive at the correct diagnosis.

Informed consent

Written informed consent was obtained from the patient.

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 patient understands that name and initials will not be published and due efforts will be made to conceal 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. Islak E. On the usual granulation combined with hyperplastic changes of lymphatic tissue. Trans Soc Fathol Jpa 1948:37:179-80.  Back to cited text no. 2
Rajesh A, Prasanth T, Naga Sirisha VC, Azmi M. Kimura's disease: A case presentation of postauricular swelling. Niger J Clin Pract 2016;19:827-30.  Back to cited text no. 3
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Dixit MP, Scott KM, Bracamonte E, Dixit NM, Schumacher MJ, Hutter J, et al. Kimura disease with advanced renal damage with anti-tubular basement membrane antibody. Pediatr Nephrol 2004;19:1404-7.  Back to cited text no. 6
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Chong WS, Thomas A, Goh CL. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: Two disease entities in the same patient: Case report and review of the literature. Int J Dermatol. 2006:45:139-145.  Back to cited text no. 14
Kung IT, Gibson JB, Bannatyne PM. Kimura's disease: A clinicopathological study of 21 cases and its distinction from angiolymphoid hyperplasia with eosinophilia. Pathology 1984;16:3944.  Back to cited text no. 15
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Chen H, Thompson LD, Aguilera NS, Abbondanzo SL. Kimura disease: A clinicopathologic study of 21 cases. Am J Surg Pathol 2004;28:50513.  Back to cited text no. 18
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. 19


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]

  [Table 1], [Table 2], [Table 3]


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