Kimura Disease 

  • Author: Karolyn A Wanat, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 11, 2010
 

Background

Kimura disease is a chronic inflammatory disorder of unknown etiology that most commonly presents as painless, unilateral cervical 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 exists 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. ALHE appears to represent an arteriovenous malformation with secondary inflammation. Kimura disease may represent a primary inflammatory process with secondary vascular proliferation.

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Pathophysiology

The pathophysiology of Kimura disease remains unknown, although an allergic reaction, trauma, and an autoimmune process have all been implicated as possible causes. Kimura disease is manifested by an abnormal proliferation of lymphoid follicles and vascular endothelium. Peripheral eosinophilia and the presence of eosinophils in the inflammatory infiltrate suggest that Kimura disease may be a hypersensitivity reaction. Some evidence has indicated that the interaction between TH 1 and TH 2 lymphocytes may result in the excessive production of eosinophilotrophic cytokines, such as interleukin 4. Persistent antigenic stimulation from insect bites, parasitic infestation, candidal infection, or viral infection may cause the activation of this cytokine pathway, but further investigation is needed.[3, 4]

Kimura disease involves the skin, lymph nodes, and salivary glands and is reported to be associated with nephrotic syndrome in approximately 15-19% of cases. The basis of this possible association is not well understood.[5, 6]

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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.[7]

Mortality/Morbidity

Kimura disease is a benign disorder with no potential for malignant transformation, but spontaneous involution is rare. The main concern is the capacity for lesions to grow and cause disfigurement. Recurrences have been reported after medical and surgical treatment of Kimura disease.

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.[8] 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.[6]

Age

Kimura disease is usually seen in young adults during the third decade of life, with the median age being 28-32 years.[8, 3, 9]

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Contributor Information and Disclosures
Author

Karolyn A Wanat, MD  Resident Physician, Department of Dermatology, University of Pennsylvania School of Medicine

Karolyn A Wanat, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and American Medical Women's Association

Disclosure: Nothing to disclose.

Coauthor(s)

Alaina J James, MD, PhD  Assistant Professor, Department of Dermatology, The Hospital of the University of Pennsylvania

Alaina J James, MD, PhD is a member of the following medical societies: American Academy of Dermatology, National Medical Association, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Takeji Nishikawa, MD  Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Daniel S Loo, MD  Associate Professor of Dermatology, Residency Program Director, Department of Dermatology, Tufts Medical Center

Daniel S Loo, MD is a member of the following medical societies: American Academy of Dermatology and Association of Professors of Dermatology

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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. 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].

  4. 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;[Medline].

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

  6. 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].

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  8. 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].

  9. 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].

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  11. 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].

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  13. 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].

  14. 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].

  15. 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].

  16. 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].

  17. 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].

  18. 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].

  19. Armstrong WB, Allison G, Pena F, Kim JK. Kimura's disease: two case reports and a literature review. Ann Otol Rhinol Laryngol. Dec 1998;107(12):1066-71. [Medline].

  20. 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].

  21. Day TA, Abreo F, Hoajsoe DK, Aarstad RF, Stucker FJ. Treatment of Kimura's disease: a therapeutic enigma. Otolaryngol Head Neck Surg. Feb 1995;112(2):333-7. [Medline].

  22. Googe PB, Harris NL, Mihm MC Jr. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: two distinct histopathological entities. J Cutan Pathol. Oct 1987;14(5):263-71. [Medline].

  23. Gumbs MA, Pai NB, Saraiya RJ, Rubinstein J, Vythilingam L, Choi YJ. Kimura's disease: a case report and literature review. J Surg Oncol. Mar 1999;70(3):190-3. [Medline].

  24. 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. Aug 1995;22(4):319-26. [Medline].

  25. 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].

  26. 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. Nov 1988;12(11):843-54. [Medline].

  27. Matsuda O, Makiguchi K, Ishibashi K, et al. Long-term effects of steroid treatment on nephrotic syndrome associated with Kimura's disease and a review of the literature. Clin Nephrol. Mar 1992;37(3):119-23. [Medline].

  28. Senel MF, Van Buren CT, Etheridge WB, Barcenas C, Jammal C, Kahan BD. Effects of cyclosporine, azathioprine and prednisone on Kimura's disease and focal segmental glomerulosclerosis in renal transplant patients. Clin Nephrol. Jan 1996;45(1):18-21. [Medline].

  29. Som PM, Biller HF. Kimura disease involving parotid gland and cervical nodes: CT and MR findings. J Comput Assist Tomogr. Mar-Apr 1992;16(2):320-2. [Medline].

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

  31. 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].

  32. Yuen HW, Goh YH, Low WK, Lim-Tan SK. Kimura's disease: a diagnostic and therapeutic challenge. Singapore Med J. Apr 2005;46(4):179-83. [Medline].

 
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