Kimura Disease: A Case Report and Review of Literature

Abstract

Kimura disease is a rare benign chronic inflammatory disorder characterised by multiple subcutaneous nodules and lymphadenopathy most commonly in head and neck region. It is seen in Asian men in their 2nd and 3rd decade. Kimura disease is usually confused for Angiolymphoid hyperplasia with eosinophilia and associated with eosinophilia and increased serum IgE. Kimura disease is also known for its recurrence. Diagnosis is by histopathological examination. Treatment is mainly surgical excision for cosmetic purposes.

Introduction

Kimura disease is an extremely rare, chronic inflammatory disorder in which lymphoid and vascular proliferation occurs. Etiology of Kimura disease still remain unclear, but the etiopathogenesis can be linked to abnormal immune response of body to prolonged antigenic stimulation by the bite of insect, viruses, neoplasm or fungus [1]. Only 200 cases have been reported worldwide based on the histopathological reports [2]. It is most commonly seen in 3rd and 4th decade [2]. Patients of Kimura disease have elevated levels of serum immunoglobulin E and have peripheral eosinophilia that might relate its etiology to hypersensitivity reaction of body against continuous antigenic stimulation [3].

Kimura disease often present as subcutaneous swelling or lymph node enlargement in head and neck region usually in preauricular, submandibular, parotid, postauricular region. It is also often accompanied by nephrotic syndrome. Kimura disease is reported mostly in Asian population although some cases have been reported in western countries also [4].

Herein we present an atypical case of Kimura disease with early prevention in 2nd decade with bilateral postauricular and preauricular swelling without renal involvement.

Case Report

A 14 year male patient from Mewat (Haryana) presented to depart of otolaryngology SMS Medical college with the complaint of swelling in right pre-auricular (Fig. 1), right post-auricular (Fig. 2), left post-auricular (Fig. 3), left suboccipital areas since 6 months, largest one being in the right pre-auricular region (2.5 * 2), which were mobile, non-tender, firm in consistency and there were no signs of inflammation seen over the swelling. Patient had significant past history of similar complaint of swellings in right pre-auricular, right post-auricular, right occipital region 3 years ago for which patient underwent excisional biopsy. Patient had no other such swellings in axillary or inguinal region patient had no hepatosplenomegaly. No history of any real disease. Blood investigations revealed normal blood count with slight eosinophilia (eosinophils-15%) with high values of Total Eosinophilic Count (TEC) 2780 cells/cumm and raised IgE (> 3000 IU/ml). USG was normal with no organomegaly, CRP and rheumatoid factor were negative and urine routine and microscopic examination was with in normal limits. Fine needle aspiration cytology suggestive of reactive hyperplasia of lymph node which did not correlate with other clinical findings so patient was subjected to excisional biopsy of the right post auricular swelling. The histopathological examination of excisional biopsy specimen showed extensive sclerotic changes with nodules of lymphoid cells, plasma cells with lymphoid hyperplasia (Fig. 4) and presence of germinal centers with large number of eosinophils seen amongst the inflammatory cells (Fig. 5) and cells showing presence of Warthin–Finkeldey type polykaryocyte (Fig. 6) suggesting chronic lymphnoditis with extensive eosinophilic infiltration and epitheloid histiocytes (Fig. 7) and was advised for immunomarker studies for final diagnosis. Further immunohistochemistry studies showed lymphoid follicles positive for CD20, CD3, CD7, CD8, CD15 and negative for CD30. All other possible diseases were excluded. Considering the age, gender, nodular pattern of the lesion in the lymph nodes with marked eosinophilic infiltration and IHC report suggested non-infectious inflammatory disorder Kimura disease. Patient was treated with steroids and managed conservatively. Patient responded well and is under regular follow ups.

Fig. 1.

Patient having left post auricular swelling

Fig. 2.

Patient having right postauricular swelling

Fig. 3.

Patient having right preauricular swelling

Fig. 4.

Lymphoid hyperplasia

Fig. 5.

Proliferation of eosinophils and mast cells

Fig. 6.

Warthin−Finkeldey type polykaryocyte (H&E × 400)

Fig. 7.

Epitheloid histiocytes along with eosinophils

Discussion

Kimura disease is a benign immune mediated chronic inflammatory disorder presents with tumor like swellings in the head and neck region with or without lymphadenopathy along with hypereosinophilia and elevated serum immunoglobulin E [5]. The first case of kimura was reported in China by Kimm and Szeto in 1937. A Japanese doctor, Kimura described this disease for the first time in 1948 hence called Kimura disease [6]. It is usually seen in men in their 3rd and 4th decade. Etiopathogenesis is not known but can be due to allergic reactions, infections or autoimmune reactions [7]. Kimura disease is seen endemic to east and only a few cases noted among Caucasians [5]. It is commonly seen in Japan, China, Philippines and occasionally the Indian subcontinent [8]. Kimura disease is most commonly confused as angiolymphoid hyperplasia with eosinophilia (ALHE) till Rosai et al. [9] did a study which clarified and established KD and ALHE as two different entities. The male-to-female ratio ranges from 3.5:1.0 to 9.0:1.0 [10].

Clinical features include painless masses in the head and neck region, eosinophilia and raised serum IgE, lymph node enlargement in axilla, groin, limbs, trunk and sometimes associated with enlargement of salivary gland [11]. Co-existing renal pathology can be there with an incidence of 10–60%, in which 10–12% patients can have nephrotic syndrome among which clinically relevant proteinuria is seen in 12–16% of patients [3]. Renal impairment is, may be, due to immunocomplex mediated damage or to Th2 dominant immune response [3]. Differential diagnosis of Kimura disease includes ALHE, Hodgkin and Non-Hodgkin lymphoma, allergic granulomatosis, Kikuchi disease, Mikulicz’s disease [5]. Histopathological examination in Kimura disease shows follicular hyperplasia with reactive germinal centers, well formed mantle zones, eosinophilic infiltrates involving the interfollicular areas of lymph nodes [11]. KD is mainly managed by surgical excision. Other than surgery systemic steroids, cytotoxic drugs, radiation therapy can also be tried [5]. Observation is often adequate in asymptomatic cases. Surgical management is mainly for cosmetic purposes as the disease is benign [6]. Intralesional injection of corticosteroids also have been shown good results [6]. Kimura’s disease is known for relapses and recurrences which is as high as 40% [12]. The overall outcome is good as it is not associated with malignancy [5].

Our patient was diagnosed as Kimura disease based on clinical features and histopathological examinaton without renal involvement was treated conservatively with immunosuppressive regimen and with regular follow up.

Conclusion

Kimura’s Disease is a rare benign chronic inflammatory disorder with multiple subcutaneous swellings and lymphadenopathy, most commonly involving head and neck region. Diagnosis is mainly by histopathological examination. The main aim of management is to maintain the cosmetic of the patient. Prognosis is good though the recurrence rate is high.

Funding

The authors received no specific funding for this work.

Compliance with Ethical Standards

Conflict of interest

The authors have no conflicts of interest to declare.

Ethical Approval

We have read and abided by the statement of ethical standards for manuscripts submitted to Indian Journal of Otolaryngology and Head and Neck Surgery.