Kimura`s disease: A rare case of axillary lymphadenopathy

Abstract

Kimura disease is a benign disorder, usually presenting with unilateral lymphadenopathy and subcutaneous nodules in the head and neck region. It has no potential for malignant transformation. Spontaneous involution is rare and the main concern is its capacity to grow and cause disfigurement. We present a case of a young female presenting with unilateral, chronic, cervical and axillary lymphadenopathy. The diagnosis of Kimura’s disease was established only on histopathology of the excised axillary lymph node.

Keywords: Kimura disease; lymphadenopathy; eosinophils; steroids

How to cite this article:
Goel A, Agarwal U, Jaiswal S S, Bhardwaj M. Kimura`s disease: A rare case of axillary lymphadenopathy. Ann Trop Med Public Health 2010;3:26-7

 

How to cite this URL:
Goel A, Agarwal U, Jaiswal S S, Bhardwaj M. Kimura`s disease: A rare case of axillary lymphadenopathy. Ann Trop Med Public Health [serial online] 2010 [cited 2020 Aug 7];3:26-7. Available from: https://www.atmph.org/text.asp?2010/3/1/26/76181

 

Introduction

Kimura disease is a chronic inflammatory disorder of unknown etiology, commonly manifesting as a painless unilateral cervical lymphadenopathy or a subcutaneous mass, in the head and neck region. The first report was from China, in 1937, in which Kimm and Szeto described seven cases of eosinophilic hyperplastic lymphogranuloma. [1] The disorder received its current name in 1948, when Kimura et al. noted the vascular component and referred to it as an unusual granulation combined with hyperplastic changes in the lymphoid tissue. [2]

Case Report

A 25-year-old female, presented to the Outpatient Department of our hospital with a complaint of swelling in the right axilla and cervical region. There was no history of pain, fever, chronic cough or loss of weight. On examination multiple lymph nodes were palpable in the right axillary and cervical region, which were firm in consistency and mobile.

The largest lymph node was about 3 cm in size. The patient was planned for lymph node biopsy. Excision of the 3 cm lymph node was performed by transverse incision in the right axilla, under local anesthesia. The external surface of the lymph node was light brown with yellowish fat. Formalin-fixed, paraffin-embedded sections of the axillary mass were stained with Hematoxylin and eosin. The histology microsections showed hyperplastic germinal centers exhibiting vascularization, interstitial fibrosis, eosinophilic infiltrations, and the formation of eosinophilic abscesses [Figure 1]. The paracortex showed hyalized vessels, with foci of sclerosis, with dense eosinophilic infiltration in the perinodal tissues [Figure 2]. Eosinophilic leucocytosis was seen on the peripheral smear, with a eosinophil count of 77%. The patient’s Ig E level was raised to 800 ΅g/I (Normal: 30 -280).

Figure 1 :Microphotograph of the lymph node showing marked follicular hyperplasia, infi ltration by eosinophils, eosinophilic abscess, and vascularization, with normal endothelial cell lining (inset).

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Figure 2 :Hematoxylin and eosin staining showing Eosinophils infiltrating the perinodal adipose tissue

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The patient was started on oral prednisolone30 mg and pentoxiphyline 400 mg, and tapered off after two months. After three months of therapy, the axillary lymph nodes regressed, the eosinophils decreased to 7%, and the total leucocyte counts decreased from 20000 to 12000.

Discussion

Kimura disease is a deep, chronic form of angiolymphatic hyperplasia, with eosinophilia. Most cases have been reported in Asians and people from the Far East. Kimura disease is limited to the skin, lymph nodes, and salivary glands, but some cases have also been reported to present in the form of a nephrotic syndrome.

Pathology of the Kimura disease is unknown, although an allergic and autoimmune process has been implicated. The disease is manifested by an abnormal proliferation of lymphoid follicles and vascular endothelium and may include peripheral eosinophilia and the presence of eosinophils in the inflammatory infiltrate. Proliferated vessels in the Kimura’s disease lack the hyaline quality, plumpness of endothelial cells, and cytoplasmic vacuoles of angiolymphoid hyperplasia with eosinophilia. The vasculature shows a normal endothelial cell lining, as compared to the epithelioid / histiocytoid endothelial cells that protrude into the vascular lumina in a ‘hobnail’ or ‘tombstone’ pattern in angiolymphoid hyperplasia with eosinophilia, helping to differentiate this entity from the latter. Serum immunoglobin E may be elevated. Blood urea nitrogen and urinary proteins should be obtained, to exclude concomitant renal dysfunction and the nephritic syndrome. Incisional biopsy is recommended for the establishment of diagnosis. Intralesional, steroid-like Trimcinolone acetonide and oral steroids can shrink nodules. In refractory cases, Cyclosporine, in a dose of 5 mg\kg\day is effective. Remission can also be achieved by combining Trans-retinoic acid with oral prednisolone. [3] Radiotherapy has been used to treat recurrent persistent lesions at dosages of 30 Gy, with success in local control, in 74% of the cases. [4] Conservative surgical excision is the treatment of choice but lesions can recur after excision.

Thus, in patients presenting with axillary lymphadenopathy with eosinophilia, Kimura’s disease should be kept in mind.

References

 

1. Kimm HT, Szeto C. Eosinophilic hyperplastic lymphogranuloma, comparison with Mikulicz’s disease. Proc Chin Med Soc 1937;329.
2. Kimura T, Yoshimura s, Ishikawa E. On the unusual granulation combined with hyperplastic changes of lymphatic tissues. Trans Soc Pathol Jpn 1948;37:179-80.
3. Boulanger E, Gachot B, Verkarre V, Valensi F, Brousse n, Hermin O. All transretinoic acid in the treatment of Kimura`s disease. Am J Hematol 2002;71:66.
4. Hareyama M, Oouchi A, Nagakura H, Asakura K, Saito A, Satoh M, et al. Radiotherapy for Kimura`s disease: the optimum dosage. Int J Radiat Oncol Biol Phys 1998;40:647-51.

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/1755-6783.76181

Figures

[Figure 1], [Figure 2]

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