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      Mepolizumab as an effective treatment for Kimura's disease associated with ulcerative colitis: A case report

      case-report

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          Abstract

          Kimura's disease was first described by Kimura and Sceto in China in 1937. Kimura disease is a chronic inflammatory disorder of idiopathic etiology. The clinical presentation of Kimura's disease is painless solitary or multiple subcutaneous nodules, asymmetric, mostly in the head and neck region with often association with lymphadenopathy. Typically, the nodules are found on preauricular, submandibular, and popliteal regions as well as oral cavity, larynx, and parotid glands. In the present report, we describe a case of a 27-year-old male presented to our hospital with history of right neck and lip swelling for 10 days. In the history, the patient mentioned that he had bloody diarrhea four to six times day and he lost 10 kg in 1 month. The specimen was sent for histopathological examination which showed the lymph node architecture is preserved with significant increase number of eosinophils which is consistent with Kimura's disease. There is no agreement on the management aspects in Kimura's disease so far. The primary treatment for Kimura's disease includes surgical resection. Additional medical therapy including regional or systemic steroid therapy, cytotoxic therapy, and radiation has also been utilized. Considered as an inflammatory process, the disease has an excellent prognosis, although it may recur locally and wax and wane over time.

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          Kimura disease: a clinicopathologic study of 21 cases.

          Kimura disease is a rare form of chronic inflammatory disorder involving subcutaneous tissue, predominantly in the head and neck region and frequently associated with regional lymphadenopathy and/or salivary gland involvement. This condition has a predilection for males of Asian descent and may clinically simulate a neoplasm. Kimura disease is sometimes confused with angiolymphoid hyperplasia with eosinophilia, which occurs in the superficial skin of the head and neck region. Although sporadic cases have been reported in non-Asians, there is no large, comprehensive study of Kimura disease in the United States. We report 21 cases with nodal involvement that, histologically, are consistent with Kimura disease. There were 18 males and 3 females (male/female ratio 6:1), 8 to 64 years of age (mean, 32 years), and included 7 Caucasians, 6 Blacks, 6 Asians, 1 Hispanic, and 1 Arabic. Anatomic sites of involvement included posterior auricular (n = 10), cervical (n = 6), inguinal (n = 3), and epitrochlear (n = 2) lymph nodes, with two patients having associated salivary gland involvement. Most (n = 16) cases had peripheral blood eosinophilia. Consistent histologic features were follicular hyperplasia, eosinophilic infiltrates, and proliferation of postcapillary venules. Follow-up data on 18 patients revealed that 13 were alive without disease (3 had recurrence), mean follow-up, 10.9 years; 4 were alive with disease (2 had a recurrence), mean follow-up, 8.8 years; and 1 died with disease (12.7 years). Kimura disease has been described more often in Asians, but it does occur in non-Asians with a similar clinicopathologic presentation. It is a distinctive entity with no known etiology. Kimura disease has characteristic histologic features that are important to recognize and can be used to differentiate it from hypersensitivity and drug reactions and infections.
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            Kimura disease: review of the literature.

            Kimura disease (KD) is a rare, chronic inflammatory disease of unknown cause and is characterized by painless s.c. swellings and lymphadenopathy commonly affecting the head and neck region. Much therapeutics has been used to treat KD, but is not satisfactory because of frequent relapse. Imatinib has been reported previously to be useful for treatment of hypereosinophilic syndrome and may work by selectively blocking protein-tyrosine kinases, such as platelet-derived growth factor receptor, and c-Kit. We carried out immunohistochemical examination of platelet-derived growth factor receptor-alpha and c-Kit in tissues from patients with KD. The results were positive and suggested that Imatinib might be an effective drug for the treatment of the disease. We have also briefly reviewed the epidemiology, aetiology, clinical manifestations, laboratory and pathological examinations, differential diagnoses, treatment and prognosis of KD in this manuscript.
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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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                Author and article information

                Journal
                J Family Med Prim Care
                J Family Med Prim Care
                JFMPC
                Journal of Family Medicine and Primary Care
                Wolters Kluwer - Medknow (India )
                2249-4863
                2278-7135
                September 2019
                30 September 2019
                : 8
                : 9
                : 3028-3031
                Affiliations
                [1 ] Department of Internal Medicine, Prince Mohammed Medical City Security, Aljouf, Riyadh, Saudi Arabia
                [2 ] Department of Internal Medicine, Security Forces Hospital (SFH), Riyadh, Saudi Arabia
                Author notes
                Address for correspondence: Dr. Abdulrahman Nasiri, Department of Internal Medicine, Security Forces Hospital (SFH), Riyadh, Saudi Arabia. E-mail: dramnasiri@ 123456gmail.com
                Article
                JFMPC-8-3028
                10.4103/jfmpc.jfmpc_373_19
                6820387
                31681687
                04a5f3e6-4ae6-46a9-a9ea-258e1cdab40f
                Copyright: © 2019 Journal of Family Medicine and Primary Care

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                : 06 May 2019
                : 06 June 2019
                : 01 July 2019
                Categories
                Case Report

                ibd,inflammatory,kimura,lung,lymphadenopathy,mepolizumab,rare,saudi arabia,ulcerative colitis

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