eMedicine Specialties > Dermatology > Lymphoma and Related Processes

Kimura Disease

Author: Karolyn A Wanat, MD, Resident Physician, Department of Dermatology, University of Pennsylvania School of Medicine
Coauthor(s): Alaina J James, MD, PhD, Assistant Professor, Department of Dermatology, The Hospital of the University of Pennsylvania
Contributor Information and Disclosures

Updated: Jan 11, 2010

Introduction

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 Szeto1 described 7 cases of a condition they termed "eosinophilic hyperplastic lymphogranuloma." The disorder received its current name in 1948, when Kimura et al2 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.

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

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

Clinical

History

Lesions of Kimura disease typically are slow-growing, painless masses with occasional pruritus of the overlying skin.

Physical

Patients with Kimura disease may present with a solitary enlarged painless lymph node or generalized lymphadenopathy. Salivary gland involvement is also frequently observed. Other findings include single or multiple pink-to-red cutaneous nodules, which are usually located on the head or neck, especially in the periauricular, parotid, or submandibular regions. Less frequently, the eyelids, orbit, and lacrimal glands may be involved. 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.

Causes

The exact etiology of Kimura disease is unknown, but it has been proposed that the interaction between TH 1 and TH 2 lymphocytes results in an abnormal production of eosinophils and immunoglobulin E. An inciting autoimmunity, allergic reaction or an alteration of immune regulation is suspected. Proposed theories include persistent antigenic stimulation following arthropod bites, parasitic infestation, or viral or candidal infection. However, none of these theories has been substantiated.3

More on Kimura Disease

Overview: Kimura Disease
Differential Diagnoses & Workup: Kimura Disease
Treatment & Medication: Kimura Disease
Follow-up: Kimura Disease
References
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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].

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

  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].

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

  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].

  12. Takeishi M, Makino Y, Nishioka H, Miyawaki T, Kurihara K. Kimura disease: diagnostic imaging findings and surgical treatment. J Craniofac Surg. Sep 2007;18(5):1062-7. [Medline].

  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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Further Reading

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Keywords

Kimura disease, Kimura’s disease, eosinophilic granuloma of soft tissue, eosinophilic hyperplastic lymphogranuloma, eosinophilic lymphofolliculosis, eosinophilic lymphofollicular granuloma, eosinophilic lymphoid granuloma

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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.

Medical Editor

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.

Pharmacy Editor

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.

Managing Editor

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.

CME Editor

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.

 
 
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