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LETTER TO EDITOR  
Year : 2021  |  Volume : 64  |  Issue : 2  |  Page : 428-429
A rare case of Kimura's disease involving parotid gland, lymph nodes, skin, and subcutaneous tissue diagnosed on frozen section


Department of Pathology, Smt. Kashibai Navale Medical College and General Hospital, Pune, Maharashtra, India

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Date of Submission23-Mar-2020
Date of Decision12-Apr-2020
Date of Acceptance04-May-2020
Date of Web Publication9-Apr-2021
 

How to cite this article:
Sinai Khandeparkar SG, Aphale V, Bharti N, Gogate BP, Solanke S. A rare case of Kimura's disease involving parotid gland, lymph nodes, skin, and subcutaneous tissue diagnosed on frozen section. Indian J Pathol Microbiol 2021;64:428-9

How to cite this URL:
Sinai Khandeparkar SG, Aphale V, Bharti N, Gogate BP, Solanke S. A rare case of Kimura's disease involving parotid gland, lymph nodes, skin, and subcutaneous tissue diagnosed on frozen section. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 May 16];64:428-9. Available from: https://www.ijpmonline.org/text.asp?2021/64/2/428/313276




Dear Editor,

A 15-year-old male presented to surgery outpatient department with an ill-defined, firm, immobile, and non-tender right preauricular swelling. It was present as a nodule since 2 years of age and had gradually increased to 3 × 2 cm extending into the concha of the pinna closely abutting the tragus. It was attached to the overlying skin with few nodular formations. Magnetic resonance imaging showed a well-defined lobulated T1 iso to hyperintense and T2/STIR hyperintense lesion seen in the subcutaneous plane measuring 4.3 × 2.8 × 4.6 cm involving the superficial lobe of the parotid gland. Radiological possibilities of vascular malformation and malignant salivary gland neoplasm were suggested. Renal and liver function tests were within normal limit.

Tissue from parotid and lymph node (LN) on frozen section (FS) showed lymphoid aggregates with prominent germinal center, vascular proliferation, and fibrosis with eosinophils [Figure 1]a and [Figure 1]b. Complete hemogram revealed relative eosinophilia with absolute eosinophil count (AEC) of 1734/μl. Diagnosis of Kimura's disease (KD) was suggested.
Figure 1: Photomicrograph of frozen section showing (a) lymphoid aggregates with prominent germinal center (H and E ×100), (b) prominent blood vessels, area of fibrosis and eosinophils (H and E ×400), Photomicrograph of lymph node showing (c) lymphoid aggregate with prominent germinal center (H and E ×100), (d) prominent blood vessels and eosinophils (H and E ×400), photomicrograph of parotid gland showing (e) involvement by Kimura's disease (H and E ×100), (f) serous acini with intercalated ducts and eosinophilic microabscess (H and E ×400), (g) Photomicrograph showing involvement of upper dermis of the skin by Kimura's disease (Hematoxylin and eosin, ×100), (h) eosinophils (Hematoxylin and eosin ×400)

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Superficial parotidectomy specimen with LN and a skin flap [Figure 1]c,[Figure 1]d,[Figure 1]e,[Figure 1]f,[Figure 1]g,[Figure 1]h sent for histopathology showed varying sized lymphoid follicles with prominent germinal centers which were well vascularized, filled with eosinophilic proteinaceous material and showed polykaryocytes and eosinophils. Interfollicular areas showed eosinophilic microabscesses and plasma cells. Vascular proliferation lined by flattened epithelium was seen. A diagnosis of KD was confirmed. Serum IgE levels were elevated (IgE = 4,250 IU/ml) further establishing the diagnosis. Postoperatively, patient was put on wysolone and azathioprine. Patient was referred to ear–nose–throat for further removal of the mass in the concha of the ear. On follow-up, AEC and serum IgE levels done after 4 months are normal.

KD is a chronic inflammatory condition seen in Asian males with an average age of 30 years.[1] The youngest patient reported is a 15 months African-American boy who had a fever, subcutaneous masses in neck and head region (HNR), asthma, eczema, and nephrotic syndrome.[2] The present case reported is in a 15-year-boy who had a preauricular swelling was asymptomatic and came for its removal due to cosmetic reasons. To the best of our knowledge, this is youngest case of KD with onset since 2 years of age documented in Indian literature.

Several theories including autoimmune, allergic, and infective components have been put forth to explain the etiology of KD.[3] It has been speculated that a viral or a parasitic trigger may alter T-cell immunoregulation or induce an IgE-mediated type 1 hypersensitivity, resulting in the release of eosinophilotrophic cytokines.[4] However, the lesion that bears the closest resemblance to KD is angiolymphoid hyperplasia with eosinophilia (ALHE), a reactive vascular hyperplasia or a vascular malformation secondary to a subcutaneous arteriovenous shunt. The misapprehension that ALHE and KD were identical or at least two ends of the same disease spectrum, has been perpetuated by their predilection for the HNR, clinical presentation as a subcutaneous mass, tendency to recur despite treatment and the presence of lymphoid infiltration with eosinophils and vascular proliferation. A study by Rosai et al. eventually established KD and ALHE as two distinct entities.[5]

Most patients present with subcutaneous lesions in the HNR, especially in the parotid with associated lymphadenopathy.[4] Although the clinical course of KD is typically progressive for several years, the disease is generally benign and self-limited, though it may be complicated by renal involvement.[6]

Fine needle aspiration cytology poses a diagnostic challenge in identifying KD which yielded insufficient material in this case.[1] In such cases FS could be a value aided alternative. In this case, LN on FS showed progressively transformed germinal center throughout the nodal tissue mimicking follicular lymphoma, vascular proliferation with prominent lymphoid population simulating paracortical hyperplasia, polykaryocytes imitating Reed Sternberg cells and immunoblasts and eosinophils the presence of which raised a suspicion for Hodgkin's lymphoma or parasitic infection. Section from parotid showed most of the histopathological features of KD. Eosinophilic microabscess which is a frequent feature was absent in the limited tissue submitted for FS. However, considering the age, duration of swelling, presence of the lymphoid collections, vascular proliferation, fibrosis, eosinophils, raised AEC, and high degree of suspicion together with awareness of this distinct entity helped us clinch the diagnosis.

ALHE, a closest differential in present case, is a distinct entity seen in women in 3rd–4th decade with no racial predilection presenting as multiple erythematous dermal papules accompanied by tenderness and pruritus. Regional lymphadenopathy, raised AEC, and serum IgE levels and tendency to develop nephrotic syndrome is not observed in ALHE. As against KD, microscopically ALHE is characterized by vessel proliferation lined by epithelioid endothelial cells, fewer lymphoid follicles, and absence of fibrosis.[5]

In asymptomatic cases, conservative observation is adequate. Surgical excision is considered the first line of treatment in symptomatic cases. KD does have a tendency to recur. Topical and systemic corticosteroids have been effective and in patient's resistant to steroids, radiation therapy has been used.[4]

Our experience with present case puts on record this rare lesion in Indian population presenting at 2 years of age, highlights the significance of awareness of this entity, high index of suspicion and the role of FS in diagnosing preauricular swelling for appropriate patient management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their 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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Dhingra H, Nagpal R, Baliyan A, Alva SR. Kimura disease: Case report and brief review of literature. Med Pharm Rep 2019;92:195-9.  Back to cited text no. 1
    
2.
AlGhamdi FE, Al-Khatib TA, Marzouki HZ, AlGarni MA. Kimura disease: No age or ethnicity limit. Saudi Med J 2016;37:315-9.  Back to cited text no. 2
    
3.
Glibbery N, Muscat K, Cascarini L. Kimura's disease of the parotid gland with cutaneous features in a Caucasian female patient. J Surg Case Rep 2018;2018:rjy067.  Back to cited text no. 3
    
4.
Park SW, Kim HJ, Sung KJ, Lee JH, Park IS. Kimura disease: CT and MR imaging findings. AJNR Am J Neuroradiol 2012;33:784-8.  Back to cited text no. 4
    
5.
Sah P, Kamath A, Aramanadka C, Radhakrishnan R. Kimura's disease - An unusual presentation involving subcutaneous tissue, parotid gland and lymph node. J Oral Maxillofac Pathol 2013;17:455-9.  Back to cited text no. 5
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6.
Ren S, Li XY, Wang F, Zhang P, Zhang Y, Li GS, et al. Nephrotic syndrome associated with Kimura's disease: A case report and literature review. BMC Nephrol 2018;19:316.  Back to cited text no. 6
    

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Correspondence Address:
Siddhi G Sinai Khandeparkar
E-517, The Island, Wakad, Pune - 411 057, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_258_20

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