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Year : 2013  |  Volume : 6  |  Issue : 2  |  Page : 208-211  

Kimura disease

Department of Pathology, Padamshree Dr. D Y Patil Medical College, Hospital & Research Centre, Dr D Y Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India

Date of Web Publication10-Apr-2013

Correspondence Address:
Shruti Vimal
D 1 702, Nisarg Nirmiti, Pimple Saudagar, Pune, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0975-2870.110319

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Kimura disease is a rare inflammatory disorder of unknown cause, primarily seen in young Asian males. The disease is characterized by painless subcutaneous swelling, blood and tissue eosinophilia. We describe a 37year old Asian male with Kimura disease who presented with multiple subcutaneous swelling. The diagnosis was based on the characteristic histopathologic findings after surgical excision in conjunction with peripheral eosinophilia.

Keywords: Eosinophilia, follicular hyperplasia, Kimura disease

How to cite this article:
Vimal S, Panicker NK, Soraisham P, Chandanwale SS. Kimura disease. Med J DY Patil Univ 2013;6:208-11

How to cite this URL:
Vimal S, Panicker NK, Soraisham P, Chandanwale SS. Kimura disease. Med J DY Patil Univ [serial online] 2013 [cited 2020 Sep 18];6:208-11. Available from:

  Introduction Top

Kimura disease is a rare chronic inflammatory disorder of unknown etiology. [1] It usually present as a deep, subcutaneous mass in the head and neck region and is frequently associated with regional lymphadenopathy or salivary gland involvement. [2] The disease was first described in the Chinese literature as "eosinophilic hyperplastic lymphogranuloma,"but became widely known as Kimura disease after Kimura et al. reported similar cases under the title "On the unusual granulation combined with hyperplastic changes of lymphatic tissue." [3] Over the years, there has been considerable confusion between Kimura disease and angiolymphoid hyperplasia with eosinophilia (ALHE). Indeed, a number of early reports used the terms Kimura disease and ALHE synonymously. It is thought, however, that the two diseases represent separate entities with distinctive clinical and histologic features. Kimura disease is considered much more prevalent in young males of Asian lineage. The nodular lesions are deep-seated in the subcutaneous tissue and clinically may mimic a neoplasm. One of the important clinicopathologic features is its frequent association with regional lymph node and/or major salivary gland involvement. Indeed, isolated lymphadenopathy might be the only initial presentation of Kimura disease. [4]

  Case Report Top

A 36-year-old male presented in surgery out-patient department with multiple subcutaneous swellings over extremities and trunk since 6 months. Initially, the swelling over the extremities were 2 mm, which then increased to the present size of 3 cm [Figure 1], with mild tenderness. Fever was absent. On examination, peripheral blood smear showed eosinophilia (20%). Axillary lymph nodes were not palpable. One of the siblings had also suffered from similar lesions in the past. With a provisional diagnosis of lipoma, an excision biopsy of largest nodule was performed and sent for histopathologic examination. The specimen showed subcutaneous fat and fascia with an ill-defined grayish white area of 2 × 1.5 × 1 cm having spots of hemorrhage. Microsections of the lesions showed diffuse and thick collections of chronic inflammatory cells, predominantly lymphocytes. Follicle formation was also seen. The background showed remarkable angioendothelial proliferation without plump epithelioid morphology. The whole lesion was infiltrated with numerous eosinophils scattered and in small groups. Fibrosis was also evident in the background. A single multinucleated giant cell was also seen. These findings shown in [Figure 2] [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], along with peripheral blood eosinophilia are typical of Kimura disease.
Figure 1: Subcutaneous swellings present over right arm and forearm

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Figure 2: Lymphoid follicle (H and E, 10×)

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Figure 3: Lymphoid follicle having dense collection of lymphocytes with eosinophils scattered and focal areas showing fibrosis (H and E, 40×)

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Figure 4: Collections of chronic inflammatory cells, predominantly lymphocytes, eosinophils, and plasmacytoid cells with few thin-walled vessels (H and E, 40×)

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Figure 5: Vasculoendothelial proliferation and eosinophils, lymphocytes, and fibrosis (H and E, 40×)

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Figure 6: Showing predominantly eosinophils with few lymphocytes, extensive fibrosis, and dilated venules. (H and E, 40×)

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Figure 7: Thin walled vessels with inflammation, predominantly eosinophils (H and E, 40×)

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Figure 8: Areas of fibrosis with variable-sized capillaries and inflammation, comprising lymphocytes and eosinophils (H and E, 40×)

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  Discussion Top

Ever since the report by Kimura et al., [3] Kimura disease has been recognized as a distinct clinicopathologic entity. Over the years, however, the disease has often been confused with ALHE, especially in the Western literature. It was assumed that the diseases were on the same spectrum due to overlapping clinicopathologic features. Clinically, both conditions present as soft tissue swellings that usually arise in the head and neck region with an indolent, prolonged clinical course. Microscopically, both processes show eosinophilic infiltrates and vascular proliferations. The confusion is escalated by the introduction of an ever-expanding number of names applied to both diseases, including eosinophilic granuloma, eosinophilic granuloma of lymph node and soft tissue, eosinophilic hyperplasic lymphogranuloma, eosinophilic lympho-follicular granuloma, eosinophilic lymphfolliculoid granuloma of the soft tissue, atypical pyogenic granuloma, inflammatory angiomatous nodules, histiocytoid hemangioma, epithelioid hemangioma, subcutaneous angioblastic lymphoid hyperplasia with eosinophilia, and subcutaneous angiolymphoid hyperplasia with eosinophilia. The first serious attempt at distinguishing Kimura disease from ALHE was done in 1979 and the latter classified under the group of histiocytoid hemangioma. [5] Others soon validated this separation with multiple comparative studies. There are characteristic and distinctive clinicopathologic features despite a few similarities between the two diseases. In brief, Kimura disease occurs commonly in young Asian males, usually presenting as deep subcutaneous cervical masses with regional lymph node and salivary gland involvement. Patients also demonstrate peripheral eosinophilia and elevated serum IgE levels. In contrast, ALHE is seen amongst all racial groups and specially amongst elderly Caucasian, with a slight female predominance. The lesions involve skin (not nodes) in the form of multiple small superficial papules, frequently erythrematous accompanied by bleeding, pruritus, and tumor growth. Regional lymphadenopathy, serum eosinophilia, and elevated Ig E levels are rare. [6],[7]

The majority of patients with Kimura disease have been reported in Asian populations in China, Taiwan, Hong Kong, and Japan. Sporadic cases of Kimura disease have been described in other ethnic groups. Subcutaneous and/or dermal involvement occurred in many of the cases. [8] The etiopathogenesis of Kimura disease is suggested as increased levels of interleukin (IL)-5 and IgE due to an abnormal T-cell stimulation, similar to a hypersensitivity reaction, [9] while others have found an association with HIV and HHV8 viral infections. [10]

Histologically, ALHE is predominantly a vasculoendothelial proliferation with formation of angioendothelial lobules having aggregates of plump endothelial cells with epithelioid morphology with some cytological atypia or cytoplasmic vacuolization. The endothelium often shows tomb stone like lining of vessel lumen. In contrast, Kimura disease has arborizing vascular proliferation of thin-walled post-capillary venules with flattened endothelial cells lacking atypia or vacuolation. Kimura disease also presents with fibrosis in the background which is not a feature of AHLE. Lymphoid proliferation and eosinophilic infiltration are common to both. Nodal and extra nodal soft tissue lymphoid follicles show folliculolysis with eosinophilic infiltration. Presence of multinucleated giant cells has been observed in Kimura lesion. [11] Infrequent small clusters of giant cells or small discrete eosinophilic necrosis with or without surrounding epithelioid histiocytes may also be seen.

Kimura disease is a chronic disorder, with an indolent clinical course, frequently waxing and waning over time. Surgery is the mainstay of therapy, although regional or systemic corticosteroid therapy, cytotoxic therapy, and radiation have been used. Recurrence after surgery or discontinued steroid treatment is common.

Although Kimura disease is a rare disorder, it should be considered in the differential diagnosis when one encounters significant eosinophilic infiltration, vasculoendothelial proliferation, and lymphoid hyperplasia in soft tissues and lymph nodes.

  References Top

1.Ioachim H, Ratech H. Kimura lymphadenopathy. In: Ioachim H, Ratech H, editors. Ioachim's Lymph Node Pathology. 3 rd ed. Philadelphia: Lippincott-Raven;, 2002. p. 209-11.  Back to cited text no. 1
2.Chan JK, Hui PK, Ng CS, Yuen NW, Kung IT, Gwi E. Epithelioid haemangioma (angiolymphoid hyperplasia with eosinophilia) and Kimura's disease in Chinese. Histopathology 1989;15:557-74.  Back to cited text no. 2
3.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. 3
4.Kuo TT, Shih LY, Chan HL. Kimura's disease: Involvement of regional lymph nodes and distinction from angiolymphoid hyperplasia with eosinophilia. Am J Surg Pathol 1988;12:843-54.  Back to cited text no. 4
5.Helander SD, Peters MS, Kuo TT, Su WP. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: New observations from immunohistochemical studies of lymphocyte markers, endothelial antigens, and granulocyte proteins. J Cutan Pathol 1995;22:319-26.  Back to cited text no. 5
6.Don DM, Ishiyama A, Johnstone AK, Fu YS, Abemayor E. Angiolymphoid hyperplasia with eosinophilia andvascular tumors of the head and neck. Am J Otolaryngol 1996;17:240-5.  Back to cited text no. 6
7.Sternberg S. Diagnostic surgical pathology. In: Mills S, Carter D, Greenson J, Reuter V, Stoler M, editor. Hematopoetic and lymphatic System; Lippincott Williams and Wilkins; 2010. p. 691.  Back to cited text no. 7
8.Chen H, Thompson LD, Aguilera N. Kimura disease: A clinicopathologic study of 21 cases. Am J Surg Pathol 2004;28;505-13.  Back to cited text no. 8
9.Rajpoot DK, Pahl M, Clark J. Nephrotic syndrome associated with Kimura disease. Pediatr Nephrol2000;14:486-8.  Back to cited text no. 9
10.Jang KA, Ahn SJ, Choi JH, Sung KJ, Moon KC, Koh JK, et al. Polymerase chain reaction (PCR) for human herpesvirus 8 and heteroduplex PCR for clonality assessment in angiolymphoid hyperplasia witheosinophilia and Kimura's disease. J Cutan Pathol 2001;28:363-7.  Back to cited text no. 10
11.Hui PK, Chan JK, Ng CS, Kung IT, Gwi E. Lymphadenopathy of Kimura's disease. Am J Surg Pathol 1989;13:177-86.  Back to cited text no. 11


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


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