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      Kimura’s disease affecting the axillary lymph nodes: a case report

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          Kimura’s disease (KD; eosinophilic granuloma of soft tissue) is an inflammatory granulomatous disorder of unknown cause with eosinophilic infiltration that occurs mainly in soft tissue. KD occurs mainly in the head and neck, but development in the axillary region is very rare.

          Case presentation

          A 74-year-old Japanese woman was evaluated for a mass that she noted in the left axillary region. On physical examination, there was a palpable, thumb-sized, hard, elastic, freely movable mass in the left axilla. Blood tests showed elevated soluble interleukin-2 receptor (sIL-2R), normal serum immunoglobulin (Ig) G4, and elevated serum IgE. Ultrasonography of the left axilla showed multiple lymph nodes (LNs) with irregular margins in which central hyperechogenicity was lost. A systemic search by computed tomography (CT) showed no systemic lymphadenopathy or other mass-like lesions suspicious for a primary tumour other than in the left axillary LNs. Biopsy of an excised LN was performed under local anaesthesia for a definitive diagnosis. Histopathology showed various-sized lymphoid follicles, large nodular lesions with an enlarged mantle zone, multiple various-sized germinal centres in single nodules, and eosinophilic infiltration between the nodes. Immunohistochemical (IHC) staining of the germinal centres was positive for cluster of differentiation (CD) 10, positive for B-cell lymphoma (bcl)-6, and negative for bcl-2. These findings led to a diagnosis of KD. Ultrasound after 3 months of follow-up showed disappearance of the axillary lymphadenopathy.


          A very rare case of KD in the axillary LNs was described. KD has the potential to occur in any region.

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          Most cited references 11

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          Kimura Disease: A Case Report and Review of the Chinese Literature

          Background: Kimura disease, often accompanied by nephrotic syndrome, is a rare, chronic inflammatory disorder of unknown cause. In this report, the clinical and histopathological characteristics of 20 Chinese patients with Kimura disease-associated nephrotic syndrome were retrospectively evaluated. Methods: We report a case of Kimura disease that was diagnosed recently in our ward, with steroid-responsive but recurrent minimal-change nephrotic syndrome. Meanwhile, we also used three powerful Chinese journal search engines (, and ScienceChina) to search the cases reported in Chinese from 1984 to 2007. Results: The nephrotic syndrome of our patient occurred 20 months after the onset of Kimura disease. Renal biopsy revealed minimal-change lesions. The patient was responsive to the steroid, but proteinuria recurred. In most of the 19 other cases, the onset of nephrotic syndrome occurred after subcutaneous masses. Renal biopsy in 13 cases showed mesangial proliferative glomerulonephritis in 9, minimal change disease in 2 and membrane nephropathy in 2 cases. Serum creatinine levels were elevated in 5 patients. Conclusion: Normally, Kimura disease-associated nephrotic syndrome patients are sensitive to prednisone therapy but are likely to relapse. In patients with recurrent nephrotic syndrome, renal insufficiency is not uncommon.
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            Kimura disease.

            Kimura disease is a benign rare chronic inflammatory disorder of unknown etiology that involves the lymph nodes and subcutaneous tissue of the head and neck regions. Elevated serum immunoglobulin E levels and peripheral blood eosinophilia are also common. This disease is most common in middle-aged Asian men. Although the etiology is unknown, it most probably represents an aberrant chronic immune response. Treatment for Kimura disease includes surgical resection and regional or systemic steroid therapy. Cytotoxic therapy and radiation have also been utilized. The disease has an excellent prognosis, although it may recur locally.
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              Lymphadenopathy of Kimura's disease.

              Kimura's disease is an important category of reactive lymphadenopathy in the Oriental population. The enlarged nodes are mostly located in the head and neck region. Salient pathological changes include florid germinal centers, Warthin-Finkeldey type polykaryocytes, vascularization of germinal centers, increased postcapillary venules in the paracortex, eosinophilic infiltration, and sclerosis. The pathology of Kimura's disease is quite different from that of angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma). Immunoperoxidase studies show IgE reticular networks in germinal centers. Nondegranulated surface IgE-positive mast cells are present in the paracortex. The authors propose that Kimura's disease represents an aberrant immune reaction to an as yet unknown stimulus. Although the individual histological features are nonspecific, the constellation of features is highly characteristic of Kimura's disease. Since lymphadenopathy can herald involvement of other tissues and the prognosis is excellent, accurate diagnosis of this disease in lymph node biopsies may spare the patients unnecessary radical surgery.

                Author and article information

                BMC Surg
                BMC Surg
                BMC Surgery
                BioMed Central (London )
                26 May 2017
                26 May 2017
                : 17
                [1 ]ISNI 0000 0004 0642 5069, GRID grid.414143.7, , Department of Surgery, Baba Memorial Hospital, ; Higashi 4-244 Hamadera Funao-cho, Nishi-ku, Sakai, Osaka 592-8555 Japan
                [2 ]ISNI 0000 0001 1009 6411, GRID grid.261445.0, Department of Surgical Oncology, , Osaka City University Graduate School of Medicine, ; 1-4-3 Asahi-machi, Abeno-ku, Osaka Japan
                [3 ]ISNI 0000 0004 1936 9967, GRID grid.258622.9, Department of Pathology, , Kinki University Faculty of Medicine, ; Osaka-sayama, Osaka 589-8511 Japan
                © The Author(s). 2017

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (, which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated.

                Funded by: FundRef, Japan Society for the Promotion of Science;
                Award ID: 26461957
                Award ID: 25461992
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                Case Report
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                © The Author(s) 2017


                biopsy, ige-rist, eosinophilic granuloma, axillary lymph nodes, kimura’s disease


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