eMedicine Specialties > Dermatology > Lymphoma and Related Processes

Kimura Disease

Author: Sena J Lee, MD, PhD, Staff Physician, Department of Dermatology, Hospital of University of Pennsylvania
Coauthor(s): Alaina J James, MD, PhD, Staff Physician, Department of Dermatology, University of Pennsylvania Medical Center; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System; Laura M Tamburin, MD, Affiliated Dermatology, PC; Patricia Mercado, MD, Chief, Dermatology Division at Birmingham Veterans Administration Medica, Associate Professor of Dermatology, Department of Dermatology, University of Alabama at Birmingham
Contributor Information and Disclosures

Updated: May 25, 2007

Introduction

Background

Kimura disease (KD) is a chronic inflammatory disorder of unknown etiology, most commonly manifesting as painless unilateral cervical lymphadenopathy or subcutaneous masses in the head or neck region. The first report of KD 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 KD and angiolymphoid hyperplasia with eosinophilia (ALHE) are the same entity. Some authors believe that KD represents a chronic deeper, form of ALHE; however, most recent papers distinguish the 2 on the basis of clinical and histopathologic characteristics. For more information, see Angiolymphoid Hyperplasia with Eosinophilia. 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 KD remains unknown, although an allergic reaction, trauma, and an autoimmune process have all been implicated as the possible cause. The 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 KD may be a hypersensitivity reaction. Some evidence has indicated that TH2 lymphocytes may also play a role, but further investigation is needed.

KD is generally limited to the skin, lymph nodes, and salivary glands, but patients with KD and nephrotic syndrome have been reported. The basis of this possible association is unclear.

Frequency

United States

KD has rarely been reported in the United States.

International

The prevalence of KD is not known, but most cases are reported from the Far East.

Mortality/Morbidity

KD 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.

Race

Most cases of KD 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 KD at the US Armed Forces Institute of Pathology found the following racial distribution: 7 whites, 6 blacks, 6 Asians, 1 Hispanic, and 1 Arab. This illustrates that if clinically suspected, KD should be included on the differential diagnosis for persons of any racial group.

Sex

Males are affected by KD more commonly than females, with a 6:1 ratio in one series.

Age

KD is usually seen in young adults. A series by Kung et al3 reported a median age of 28 years.

Clinical

History

Lesions of KD typically are slowly enlarging painless masses with occasional pruritus of the overlying skin.

Physical

Patients 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 subcutaneous 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 KD mainly affects the head and neck, involvement of the extremities and inguinal lymph nodes has been reported.

Causes

The cause of KD is unknown; however, an allergic reaction or an alteration of immune regulation is suspected. Proposed theories include persistent antigenic stimulation following arthropod bites and parasitic or candidal infection. To date, none of these theories has been substantiated.

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. 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].

  4. 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].

  5. 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].

  6. 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].

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

  8. 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].

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

  12. 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].

  13. 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].

  14. 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].

  15. 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].

  16. 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].

  17. Matsuda O, Makiguchi K, Ishibashi K, Chida Y, Ida T, Matsuda 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].

  18. 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].

  19. 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].

  20. 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].

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

  22. 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].

  23. 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

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

Sena J Lee, MD, PhD, Staff Physician, Department of Dermatology, Hospital of University of Pennsylvania
Sena J Lee, MD, PhD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Alaina J James, MD, PhD, Staff Physician, Department of Dermatology, University of Pennsylvania Medical Center
Disclosure: Nothing to disclose.

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other

Laura M Tamburin, MD, Affiliated Dermatology, PC
Laura M Tamburin, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Patricia Mercado, MD, Chief, Dermatology Division at Birmingham Veterans Administration Medica, Associate Professor of Dermatology, Department of Dermatology, University of Alabama at Birmingham
Patricia Mercado, MD is a member of the following medical societies: American Academy of Dermatology and American Medical Association
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, Residency Program Director, Department of Dermatology, Boston University School of Medicine
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: Nothing to disclose.

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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