Background

Kimura disease is a chronic inflammatory disorder of unknown etiology that most commonly presents as painless lymphadenopathy or subcutaneous masses in the head or neck region. The first report of Kimura disease was from China in 1937, in which Kimm and Szeto[1] described 7 cases of a condition they termed "eosinophilic hyperplastic lymphogranuloma." The disorder received its current name in 1948, when Kimura et al[2] noted the vascular component and referred to it as an "unusual granulation combined with hyperplastic changes in lymphoid tissue."

Controversy has existed in the literature regarding whether Kimura disease and angiolymphoid hyperplasia with eosinophilia (ALHE) are the same entity. Some authors believe that Kimura disease represents a chronic, deeper form of ALHE; however, most recent papers distinguish the 2 on the basis of clinical and histopathologic characteristics.[3] ALHE appears to represent an arteriovenous malformation with secondary inflammation. Kimura disease may represent a primary inflammatory process with secondary vascular proliferation.

Pathophysiology

The pathophysiology of Kimura disease remains unknown. It has been hypothesized that an infection or toxin may trigger an autoimmune phenomenon or lead to a type I (immunoglobulin E [IgE]–mediated) hypersensitivity reaction. Some evidence has suggested a predominance of TH

2 cells (which produce eosinophilic cytokines, including interleukin [IL]–4 and IL-5) in patients with Kimura disease.[4] Additional studies have shown elevated granulocyte macrophage-stimulating factor (GM-CSF), tumor necrosis factor-a (TNF-a), soluble IL-2 receptor (sIL-2R), IL-5, IL-4, and IL-13.[5] These findings may help lay the groundwork for elucidating the underlying pathophysiology of Kimura disease.

Epidemiology

Frequency

United States

Kimura disease has rarely been reported in the United States.

International

The exact prevalence of Kimura disease is not known. Most cases of this rare disease are reported in East and Southeast Asia, with a small number of cases reported in Europe.[6]

Mortality/Morbidity

Kimura disease can lead to disfigurement secondary to the growth of untreated lesions, particularly given the predilection for the head and neck. Additionally, recurrence after treatment is well described.

Race

Most cases of Kimura disease have been reported in Asians, and the prevalence among persons of other races is thought to be low. A retrospective review of 21 histopathologic specimens diagnosed as Kimura disease at the US Armed Forces Institute of Pathology found the following racial distribution: 7 whites, 6 African Americans, 6 Asians, 1 Hispanic, and 1 Arab.[7] This illustrates that if clinically suspected, Kimura disease should be included on the differential diagnosis for persons of any racial group.

Sex

Males are affected by Kimura disease more commonly than females, with a 3.5:1 to 9:1 male-to-female ratio in most series reported, with the exception of one series in which the male-to-female ratio was 19:1.[8]

Age

Kimura disease is usually seen in young adults during the third decade of life, with the median age being 28-32 years.[7, 9, 10]

History

Kimura disease typically presents as a painless mass or masses in the head and neck region, with occasional pruritus of the overlying skin. Renal disease, nephrotic syndrome in particular, is present in up to 20% of patients with Kimura disease.[11]

Physical

Patients typically present with nontender subcutaneous nodules and masses in the head and neck, especially in the parotid and submandibular regions. These lesions are typically associated with lymphadenopathy. Less frequently, the orbit (including the eyelids, conjunctiva, and lacrimal glands[12] ), paranasal sinuses, epiglottis, tympanic membrane, and parapharyngeal space may be involved.[13] The average diameter of lesions is 3 cm. Although Kimura disease mainly affects the head and neck, involvement of the extremities and inguinal lymph nodes has been reported.[14]

Differential Diagnoses

Angiolymphoid Hyperplasia with Eosinophilia Cylindroma Dermatofibrosarcoma Protuberans Kaposi Sarcoma Pyogenic Granuloma (Lobular Capillary Hemangioma)

Laboratory Studies

Nearly all patients with Kimura disease demonstrate peripheral eosinophilia and elevated levels of serum IgE. In one series, the number of eosinophils was closely correlated to the sizes of the neck masses.[15]

Blood urea nitrogen, creatinine, and urinary protein levels should be obtained to exclude concomitant renal dysfunction (especially nephrotic syndrome).

Serum eosinophil cationic protein levels parallel the course of the disease.[16]

Imaging Studies

The appearance of Kimura disease on imaging modalities, including CT scanning and MRI, is variable and is thought to be due, at least in part, to the variable degrees of vascular proliferation and fibrosis within individual lesions. One of the largest case series to date notes the characteristic findings to be multiple ill-defined, enhancing lesions around the parotid gland, with associated lymphadenopathy.[13]

Procedures

Incisional biopsy is recommended to obtain the diagnosis of Kimura disease.

Histologic Findings

Lymphoid nodules with discrete germinal centers can occupy an area extending from the reticular dermis to the fascia and muscle. A marked eosinophilic infiltrate and eosinophilic abscesses are present. Centrally, thick-walled vessels are present with hobnail endothelial cells. Immunohistochemical evaluation of the lymphoid nodules demonstrates a polymorphous infiltrate without clonality.[6, 13] Reports have also demonstrated the presence of plasmacytoid dendritic cells in a lesion of Kimura disease.[17]

Medical Care

Observation is acceptable if the Kimura disease lesions are neither symptomatic nor disfiguring.

Oral corticosteroids are commonly used; however, the disease frequently recurs after cessation of therapy. Intralesional corticosteroids may be effective for localized disease.

Cyclosporine has been reported to induce remission in patients with Kimura disease.[18, 19] A dose of 5 mg/kg/d was effective, but, in most cases, the lesions recurred upon cessation of therapy.[20]

Intravenous immunoglobulin (IVIG) was used in one patient as a steroid-sparing agent, and he remained disease free more than 6 years after follow-up.[21]

Oral pentoxifylline has been reported to be effective in one patient with Kimura disease; however, the lesions relapsed after discontinuation of therapy.[22]

All trans -retinoic acid in combination with prednisone has resulted in remission of Kimura disease in one patient, and he remained disease free 12 months after discontinuation of all therapy.[23]

Imatinib may be an effective treatment for Kimura disease, based on advances in research for therapy in hypereosinophilic syndrome, but further investigation is necessary.[6]

Photodynamic therapy has been used successfully in one patient who experienced recurrence of disease after initial surgical management.[24]

Radiotherapy has occasionally been used to treat recurrent or persistent Kimura disease lesions. A report by Hareyama et al[25] reported on the use of radiotherapy at dosages of 26-30 Gy; local control was achieved in 74% of lesions. Another study demonstrated that radiotherapy (20-45 Gy) was more effective than local excision and steroid treatment, with local response rates of 64.3% versus 22.2%, respectively. No adverse effects were observed during a mean follow-up period of 65 months.[26] However, considering the benign nature of Kimura disease, further investigation may be required, and caution using radiation outside of recurrent, disfiguring lesions is required.

Surgical Care

Conservative surgical excision has been considered the treatment of choice for Kimura disease.[27] Recurrence after surgery is frequently observed.

Consultations

Consultation with an otolaryngologist or ophthalmologist should be considered for further evaluation depending on the extent and location of the disease.

Medication Summary

The goals of pharmacotherapy for Kimura disease are to reduce morbidity and to prevent complications.

Immunosuppressants

Class Summary

Immunosuppressants suppress the response of the immune system to diverse stimuli.

Cyclosporine (Sandimmune, Neoral)

Cyclosporine has been demonstrated to be helpful in a variety of skin disorders. It is a cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions, such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft versus host disease for a variety of organs. For children and adults, base dosing on ideal body weight.

Triamcinolone (Amcort, Aristocort)

Triamcinolone is used for inflammatory dermatosis responsive to steroids. It decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability. Intralesional injections may be used for localized skin disorders.

Prednisone (Orasone, Deltasone, Meticorten, Sterapred)

Prednisone may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear leukocyte activity.

Hemorheologic agents

Class Summary

These agents are used to treat vascular disease.

Pentoxifylline (Pentoxil, Trental)

Pentoxifylline may alter rheology of red blood cells, which, in turn, reduces blood viscosity.

Retinoids

Class Summary

These agents regulate cell growth and differentiation.

Tretinoin (Vesanoid)

Tretinoin may inhibit granulocyte differentiation.

Complications

Rarely, large nodules or tumors from Kimura disease have ulcerated. After treatment, recurrence also has been reported.

Abbas et al reported one case of postsurgical facial disfigurement effectively treated with photodynamic therapy.[24]

Prognosis

The course of Kimura disease is chronic, with lesions frequently persisting or recurring despite treatment.

To date, malignant transformation of Kimura disease has not been described in the literature.

If not treated, Kimura disease can lead to disfigurement.

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. Ramchandani PL, Sabesan T, Hussein K. Angiolymphoid hyperplasia with eosinophilia masquerading as Kimura disease. Br J Oral Maxillofac Surg. Jun 2005;43(3):249-52. [Medline].

4. Ohta N, Fukase S, Suzuki Y, Ito T, Yoshitake H, Aoyagi M. Increase of Th2 and Tc1 cells in patients with Kimura's disease. Auris Nasus Larynx. Feb 2011;38(1):77-82. [Medline].

5. Katagiri K, Itami S, Hatano Y, Yamaguchi T, Takayasu S. In vivo expression of IL-4, IL-5, IL-13 and IFN-gamma mRNAs in peripheral blood mononuclear cells and effect of cyclosporin A in a patient with Kimura's disease. Br J Dermatol. Dec 1997;137(6):972-7. [Medline].

6. Sun QF, Xu DZ, Pan SH, et al. Kimura disease: review of the literature. Intern Med J. Aug 2008;38(8):668-72. [Medline].

7. Kung IT, Gibson JB, Bannatyne PM. Kimura's disease: a clinico-pathological study of 21 cases and its distinction from angiolymphoid hyperplasia with eosinophilia. Pathology. Jan 1984;16(1):39-44. [Medline].

8. Wang DY, Mao JH, Zhang Y, et al. Kimura disease: a case report and review of the Chinese literature. Nephron Clin Pract. 2009;111(1):c55-61. [Medline].

9. Thomas J, Jayachandran NV, Chandrasekhara PK, Rajasekhar L, Narsimulu G. Kimura's disease--an unusual cause of lymphadenopathy in children. Clin Rheumatol. May 2008;27(5):675-7. [Medline].

10. Chen H, Thompson LD, Aguilera NS, Abbondanzo SL. Kimura disease: a clinicopathologic study of 21 cases. Am J Surg Pathol. Apr 2004;28(4):505-13. [Medline].

11. Rajpoot DK, Pahl M, Clark J. Nephrotic syndrome associated with Kimura disease. Pediatr Nephrol. Jun 2000;14(6):486-8. [Medline].

12. Yoganathan P, Meyer DR, Farber MG. Bilateral lacrimal gland involvement with Kimura disease in an African American male. Arch Ophthalmol. Jun 2004;122(6):917-9. [Medline].

13. Park SW, Kim HJ, Sung KJ, Lee JH, Park IS. Kimura disease: CT and MR imaging findings. AJNR Am J Neuroradiol. Apr 2012;33(4):784-8. [Medline].

14. Yadla M, Sriramnaveen P, Sivakumar V, Sandeep Reddy Y, Sridhar AV, Krishna Kishore C. Epitrochlear mass in a patient on maintenance hemodialysis-Kimura disease. Hemodial Int. Jan 26 2012;[Medline].

15. Sakamoto M, Komura A, Nishimura S. Hematoserological analysis of Kimura's disease for optimal treatment. Otolaryngol Head Neck Surg. Jan 2005;132(1):159-60. [Medline].

16. Ohta N, Okazaki S, Fukase S, Akatsuka N, Aoyagi M, Yamakawa M. Serum concentrations of eosinophil cationic protein and eosinophils of patients with Kimura's disease. Allergol Int. Mar 2007;56(1):45-9. [Medline].

17. Dargent JL, Vannuffel P, Saint-Remy JM, Fisogni S, Facchetti F. Plasmacytoid dendritic cells in Kimura disease. Am J Dermatopathol. Dec 2009;31(8):854-6. [Medline].

18. Birol A, Bozdogan O, Keles H, et al. Kimura's disease in a Caucasian male treated with cyclosporine. Int J Dermatol. Dec 2005;44(12):1059-60. [Medline].

19. Wang YS, Tay YK, Tan E, Poh WT. Treatment of Kimura's disease with cyclosporine. J Dermatolog Treat. 2005;16(4):242-4. [Medline].

20. Kaneko K, Aoki M, Hattori S, Sato M, Kawana S. Successful treatment of Kimura's disease with cyclosporine. J Am Acad Dermatol. Nov 1999;41(5 Pt 2):893-4. [Medline].

21. Hernandez-Bautista V, Yamazaki-Nakashimada MA, Vazquez-Garcia R, Stamatelos-Albarran D, Carrasco-Daza D, Rodriguez-Lozano AL. Treatment of Kimura disease with intravenous immunoglobulin. Pediatrics. Dec 2011;128(6):e1633-5. [Medline].

22. Hongcharu W, Baldassano M, Taylor CR. Kimura's disease with oral ulcers: response to pentoxifylline. J Am Acad Dermatol. Nov 2000;43(5 Pt 2):905-7. [Medline].

23. Boulanger E, Gachot B, Verkarre V, Valensi F, Brousse N, Hermine O. all-trans-Retinoic acid in the treatment of Kimura's disease. Am J Hematol. Sep 2002;71(1):66. [Medline].

24. Abbas S, Jerjes W, Upile T, Vincent A, Hopper C. Treatment of Kimura disease with photodynamic therapy: a case study. Photodiagnosis Photodyn Ther. Mar 2012;9(1):83-6. [Medline].

25. Hareyama M, Oouchi A, Nagakura H, et al. Radiotherapy for Kimura's disease: the optimum dosage. Int J Radiat Oncol Biol Phys. Feb 1 1998;40(3):647-51. [Medline].

26. Chang AR, Kim K, Kim HJ, Kim IH, Park CI, Jun YK. Outcomes of Kimura's disease after radiotherapy or nonradiotherapeutic treatment modalities. Int J Radiat Oncol Biol Phys. Jul 15 2006;65(4):1233-9. [Medline].

27. Kapoor NS, O'Neill JP, Katabi N, Wong RJ, Shah JP. Kimura disease: diagnostic challenges and clinical management. Am J Otolaryngol. Mar 2012;33(2):259-62. [Medline].

Penyakit Kimura, Gangguan Inflamasi Hipereaktifitas Kronis Jinak

Penyakit Kimura adalah gangguan inflamasi kronis jinak yang sangat jarang terjadi. Gejala utamanya adalah lesi subdermal di kepala atau leher atau peradangan sepihak tanpa rasa sakit kelenjar getah bening leher rahim. Gejala benjolan di lengan atas kanan dekat sendi siku dengan hasil pemeriksaan histopatologis penyakit Kimura.

Penyakit Kimura atau Angiolymphoid Hyperplasia dengan Eosinophilia adalah gangguan inflamasi kronis etiologi yang tidak diketahui yang paling sering muncul sebagai rasa sakit, limfadenopati servikal unilateral atau massa subkutan di daerah kepala atau leher.

Penyakit Kimura sebaiknya disertakan dalam diagnosis banding pada pasien dengan benjolan unilateral yang tidak nyeri,khususnya bila didapati di daerah leher dan atau tungkai.  Adanya eosinofilia,hyperimmunoglobuliemia (IgE) menentukan perbedaan dengan kelainan yang hampir serupa. Prognosisnya baik dan belum dijumpai keganasan.

Laporan pertama penyakit Kimura adalah dari daratan Cina pada tahun 1937, ketika Kimm dan Szeto mengidentifikasi tujuh kasus kondisi tersebut.  Nama penyakit tersebut semakin dikenal pada tahun 1948 ketika Kimura dan lain-lain mencatat perubahan dalam pembuluh darah sekitarnya dan menyebutnya sebagai granulasi dikombinasikan dengan perubahan hiperplastik pada jaringan limfoid.

Penyebab

Penyebab penyakit Kimura masih belum diketahui. Diduga karena reaksi alergi atau perubahan peraturan kekebalan tubuh. Teori lain seperti stimulasi antigen yang persisten setelah gigitan arthropoda dan infeksi parasit atau candida juga telah diusulkan. Sampai saat ini, tidak satupun dari teori telah dibuktikan.

Kontroversi yang ada dalam literatur mengenai apakah penyakit Kimura dan hiperplasia angiolymphoid dengan eosinofilia (ALHE) adalah entitas yang sama. Beberapa penulis percaya bahwa penyakit Kimura merupakan bentuk kronis pada ALHE, namun, makalah terbaru membedakan berdasarkan karakteristik klinis dan histopatologis. ALHE tampaknya berasal malformasi arteriovenosa dengan peradangan sekunder. Kimura penyakit mungkin merupakan proses inflamasi primer dengan proliferasi vaskuler sekunder.

Patofisiologi

Patofisiologi penyakit Kimura tetap tidak diketahui, meskipun reaksi alergi, trauma, dan proses autoimun semuanya telah terlibat sebagai kemungkinan penyebab.

Penyakit Kimura dimanifestasikan oleh proliferasi abnormal dari folikel limfoid dan endotelium vaskular. Peripheral eosinofilia dan adanya eosinofil pada inflamasi menyusup menunjukkan bahwa penyakit Kimura mungkin merupakan reaksi hipersensitivitas. Beberapa bukti telah menunjukkan bahwa interaksi antara TH 1 dan TH 2 limfosit dapat menyebabkan produksi yang berlebihan sitokin eosinophilotrophic, seperti interleukin 4.

Antigenik stimulasi terus-menerus dari gigitan serangga, infeksi parasit, infeksi kandida, atau infeksi virus dapat menyebabkan aktivasi dari jalur sitokin, namun penyelidikan lebih lanjut diperlukan

Penyakit Kimura  melibatkan kulit, kelenjar getah bening, dan kelenjar ludah dan dilaporkan dikaitkan dengan sindrom nefrotik pada sekitar 15-19% kasus. Dasar dari hubungan ini mungkin belum dipahami dengan baik

Penyakit ini ditunjukkan oleh proliferasi abnormal dari folikel limfoid dan endotelium vaskular. Peripheral eosinofilia dan adanya eosinofil pada inflamasi menyusup menunjukkan bahwa Penyakit Kimura mungkin merupakan reaksi hipersensitivitas.

Penyakit Kimura ini umumnya terbatas pada kulit, kelenjar getah bening, dan kelenjar ludah, tetapi pasien dengan Penyakit Kimura dan sindrom nefrotik telah dilaporkan. Dasar dari asosiasi ini mungkin tidak jelas

Interaksi antara TH 1 dan TH 2 limfosit hasil dalam produksi abnormal dari eosinofil dan immunoglobulin E. Sebuah reaksi autoimunitas, reaksi alergi atau perubahan peraturan kekebalan tubuh diduga sebagai penyebab. Teori yang diusulkan meliputi stimulasi antigen yang persisten setelah gigitan arthropoda, infestasi parasit, atau infeksi virus atau candida. Namun, tidak satupun dari teori-teori ini telah dibuktikan

Epidemiologi

Frekuensi dan Distribusi Kimura Penyakit ini terutama terlihat pada laki-laki keturunan Asia. Penyakit ini jarang dilaporkan di Amerika Serikat. Pada tingkat internasional, prevalensi penyakit ini tidak diketahui

Manifestasi Klinis

Lesi penyakit Kimura biasanya lambat tumbuh, massa tanpa rasa sakit dengan pruritus sesekali kulit di atasnya.

Penyakit Kimura ditandai dengan kelenjar getah bening soliter membesar tanpa rasa sakit atau limfadenopati generalisata.

Keterlibatan kelenjar ludah juga sering diamati. Temuan lainnya termasuk nodul kulit satu atau beberapa warna pink ke merah, yang biasanya terletak di kepala atau leher, terutama di wilayah periauricular, parotis, submandibula atau.

Agak jarang didapatkan kelopak mata, orbit, dan kelenjar lakrimal. Rata-rata diameter lesi adalah 3 cm. Meskipun Kimura penyakit terutama mempengaruhi

kepala dan leher, keterlibatan ekstremitas dan kelenjar getah bening inguinal telah dilaporkan.

Diagnosis Banding

Angiolymphoid Hyperplasia with Eosinophilia Cylindroma Dermatofibrosarcoma Protuberans Kaposi Sarcoma Pyogenic Granuloma (Lobular Capillary Hemangioma)

Pengobatan

Steroid intralesi atau oral dapat mengecilkan nodul tetapi jarang menghasilkan kesembuhan.

Siklosporin telah dilaporkan untuk menginduksi remisi pada pasien dengan Penyakit Kimura. Namun, kekambuhan dari lesi telah diamati sekali terapi ini dihentikan.

Cetirizine adalah agen efektif dalam mengobati gejala penyakit Kimura. Sifat cetirizine itu menjadi efektif baik dalam pengobatan pruritus (gatal) dan sebagai agen anti-inflamasi membuatnya cocok untuk pengobatan dari pruritus yang terkait dengan lesi

Dalam sebuah studi tahun 2005, American College of Rheumatology dilakukan peengobatan awal menggunakan prednison, diikuti dengan dosis steroid dan azathioprine, omeprazol, dan kalsium dan vitamin D selama dua tahun. Kondisi kulit pasien mulai membaik dan kulit lesi berkurang. Namun, ada gejala hirsutisme cushingoid dan diamati sebelum pasien telah dihapus dari program. Jumlah steroid diguinakan 10 mg / hari cetirizine untuk mencegah lesi kulit sekaligus untuk pengobatan pruritus berhubungan dengan lesi tersebut. Tberbagai gejala membaik, kulit pasien lesi menghilang setelah pengobatan dengan cetirizine, darah eosinofil jumlah menjadi normal, efek kortikosteroid berkurang dan remisi mulai dalam waktu dua bulan.

Radioterapi telah digunakan untuk mengobati lesi berulang atau berkelanjutan. Sebuah laporan oleh Hareyama dkk melaporkan penggunaan radioterapi pada dosis 26-30 Gy; kontrol lokal dicapai pada 74% dari lesi. Studi lain menunjukkan bahwa radioterapi (20-45 Gy) lebih efektif daripada eksisi lokal dan pengobatan steroid, dengan tingkat respon lokal dari 64,3% vs 22,2%, masing-masing. Tidak ada efek samping yang diamati selama periode tindak lanjut rata-rata 65 bulan. Namun, mengingat sifat jinak penyakit Kimura, penyelidikan lebih lanjut mungkin diperlukan, dan hati-hati menggunakan radiasi luar berulang, menodai lesi diperlukan.Siklosporin telah dilaporkan untuk menginduksi remisi pada pasien dengan penyakit Kimura. Sebuah dosis 5 mg / kg / hari efektif, namun, dalam banyak kasus, lesi terulang pada penghentian terapi.

Pentoxifylline oral telah dilaporkan efektif pada satu pasien dengan penyakit Kimura;. Namun, lesi kambuh setelah penghentian terapi

Semua asam trans-retinoic dalam kombinasi prednison telah menghasilkan remisi penyakit Kimura pada satu pasien, dan ia tetap bebas penyakit 12 bulan setelah penghentian terapi semua.

Imatinib mungkin merupakan pengobatan yang efektif untuk penyakit Kimura, berdasarkan kemajuan dalam penelitian untuk terapi pada sindrom berapapun, tetapi penyelidikan lebih lanjut diperlukan.

Penghambatan eosinofil dapat menjadi kunci untuk pengobatan penyakit Kimura karena peran eosinofil, bukan sel-sel lain berkaitan dengan lesi kulit.

Radioterapi telah digunakan untuk mengobati lesi berulang atau persisten. Namun, mengingat sifat jinak penyakit ini, radiasi harus dipertimbangkan hanya dalam kasus-kasus berulang, menodai lesi.

Bedah telah dianggap sebagai terapi utama. Namun, kekambuhan setelah operasi sering terjadi

Karena keterlibatan eosinofil dan hipereaktifitas diduga pengendalian gejala alergi lainnya dapat mengendalikan kekambuhan penyakit ini

Farmakoterapi

Tujuan dari farmakoterapi untuk penyakit Kimura adalah untuk mengurangi morbiditas dan mencegah komplikasi.

Imunosupresan  Menekan respon sistem kekebalan tubuh terhadap rangsangan beragam.

Siklosporin (Sandimmune, Neoral)  Menunjukkan untuk membantu dalam berbagai gangguan kulit.

Siklik polipeptida yang menekan beberapa imunitas humoral dan, pada tingkat yang lebih besar, sel-dimediasi reaksi kekebalan tubuh, seperti hipersensitivitas tertunda, penolakan allograft, encephalomyelitis alergi eksperimental, dan penyakit graft versus host untuk berbagai organ. Untuk anak-anak dan orang dewasa, dasar pemberian dosis pada berat badan ideal.

Triamcinolone (Amcort, Aristocort)  Untuk inflamasi dermatosis yang responsif terhadap steroid. Mengurangi inflamasi dengan menekan migrasi leukosit polimorfonuklear dan permeabilitas kapiler membalikkan. Suntikan intralesi dapat digunakan untuk gangguan kulit lokal.

Prednisone (Orasone, Deltasone, Meticorten, Sterapred)  Dapat menurunkan peradangan dengan membalikkan peningkatan permeabilitas kapiler dan menekan aktivitas PMN.

Hemorheologic digunakan untuk mengobati penyakit pembuluh darah.

Pentoxifylline (Pentoxil, Trental)  Dapat mengubah reologi sel darah merah, yang, pada gilirannya, mengurangi kekentalan darah

Retinoid  mengatur pertumbuhan sel dan diferensiasi.

Tretinoin (Vesanoid)  Dapat menghambat diferensiasi granulosit.

Referensi:

Karolyn A Wanat, Dirk M Elston. Kimura Disease. http://emedicine.medscape.com/article/1098777-overview

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.

Thomas J, Jayachandran NV, Chandrasekhara PK, Rajasekhar L, Narsimulu G. Kimura’s disease–an unusual cause of lymphadenopathy in children. Clin Rheumatol. May 2008;27(5):675-7.

Kimm HT, Szeto C. Eosinophilic hyperplastic lymphogranuloma, comparison with Mikulicz’s disease. Proc Chin Med Soc. 1937;329.

Sun QF, Xu DZ, Pan SH, et al. Kimura disease: review of the literature. Intern Med J. Aug 2008;38(8):668-72.

Kung IT, Gibson JB, Bannatyne PM. Kimura’s disease: a clinico-pathological study of 21 cases and its distinction from angiolymphoid hyperplasia with eosinophilia. Pathology. Jan 1984;16(1):39-44.

Chen H, Thompson LD, Aguilera NS, Abbondanzo SL. Kimura disease: a clinicopathologic study of 21 cases. Am J Surg Pathol. Apr 2004;28(4):505-13.

Masayuki S, Ayako K, Shinichi N. Hematoserological analysis of Kimura’s disease for optimal treatment. Otolaryngol Head Neck Surg. 2005;132:159-160.

Mrówka-Kata K, Kata D, Kyrcz-Krzemien S, Helbig G. Kikuchi-Fujimoto and Kimura diseases: the selected, rare causes of neck lymphadenopathy. Eur Arch Otorhinolaryngol. Oct 16 2009;

Rajpoot DK, Pahl M, Clark J. Nephrotic syndrome associated with Kimura disease. Pediatr Nephrol. Jun 2000;14(6):486-8.

Wang DY, Mao JH, Zhang Y, et al. Kimura disease: a case report and review of the Chinese literature. Nephron Clin Pract. 2009;111(1):c55-61.

Ohta N, Okazaki S, Fukase S, Akatsuka N, Aoyagi M, Yamakawa M. Serum concentrations of eosinophil cationic protein and eosinophils of patients with Kimura’s disease. Allergol Int. Mar 2007;56(1):45-9.

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