Kikuchi's Disease

Kikuchi's Disease

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Kikuchi's Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Histiocytic Necrotizing Lymphadenitis
  • HNL
  • Kikuchi's Histiocytic Necrotizing Lymphadenitis
  • Kikuchi-Fujimoto Disease
  • Necrotizing Lymphadenitis

Disorder Subdivisions

  • None

General Discussion

Kikuchi's disease, also known as histiocytic necrotizing lymphadenopathy, is a rare, benign, (noncancerous, nonmalignant) disorder of the lymph nodes of young adults, predominantly of young women. This disorder is often mistaken for malignant lymphoma, especially cervical adenopathy because the symptoms are very similar. The lesions, or tissue anomalies, of this disorder cause the lymph nodes to become enlarged (lymphadenopathy), inflamed and painful. The exact cause of Kikuchi's disease is not known. Perhaps the primary threat is a misdiagnosis of a malignant lymphoma.

Symptoms

Kikuchi's disease is a rare nonmalignant disorder that affects the lymph nodes. Lymph nodes are present throughout the body as small oval structures that filter lymph fluid, fight infection and form white blood cells and blood plasma cells. In addition to swelling and pain in the abdomen the lymph nodes on the side of the neck and near the salivary glands are often be affected.



Affected individuals may develop mild fever, night sweats, muscle pain (myalgia) and a rash. Less common symptoms include headaches, fatigue, joint pain (arthralgia), and nausea and vomiting. In some cases, affected individuals may experience abnormal enlargement of the liver or spleen (hepatosplenomegaly). The symptoms of Kikuchi's disease may develop slowly over two to three weeks.



For reasons that are unclear the abnormal tissue growth and inflammation in Kikuchi's disease usually clears up, spontaneously, within a few weeks or months without further therapy (self-limited disease).

Causes

The exact cause of Kikuchi's disease is not known (idiopathic). Many researchers suspect that a virus may cause this disorder. Others, however, believe that the disorder, like systemic lupus erythematosus, is a self-limited autoimmune condition. Autoimmune disorders are caused when the body's natural defenses against "foreign" or invading organisms begin to attack healthy tissue for unknown reasons.

Affected Populations

Kikuchi's disease, first identified in Japan during 1972, is extremely rare with fewer than 50 cases reported in the medical literature. It appears to affect young adults particularly, but can occur at any age. Early reports suggest that Kikuchi's disease affects women more often than men by a 4:1 ratio. Recently reports suggest that the ratio is not that large. Kikuchi's disease occurs with greater frequency in Southeast Asia.



Kikuchi's disease may be more prevalent than has been previously recognized since swollen glands are common and frequently ignored. This, combined with the self-limiting nature of the disorder, has led some clinicians to speculate that Kikuchi's disease is more common than originally thought. Since this disorder can only be identified unequivocally by biopsy of the tissue in the affected lymph nodes, it may be overlooked by many unsuspecting individuals and physicians.

Standard Therapies

Treatment of Kikuchi's disease is symptomatic and supportive. Usually the disorder resolves itself spontaneously within a few weeks or months. Analgestics-antipyretics and nonsteroidal anti-inflammatory (NSAIDs) may be used to treat pain, tenderness and fever associated with lymphadenopathy. In extremely rare cases, Kikuchi's disease may recur.



Kikuchi's disease is diagnosed by removing tissue with a fine needle that is attached to a syringe. The tissue is then examined under a microscope to determine whether the patient has the disease.

Investigational Therapies

All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (800) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOK

Huntington DS, et al. Atypical immune proliferations. In: Hoffman R, et al., eds. Hematology. New York, NY: Churchill Livingstone, Inc; 1995:1395.



REVIEW ARTICLES

Bosch X, Guilabert A. Kikuchi-Fujimoto disease. Orphanet J Rare Dis. 2006;1:18.



Lozano Parras MA, et al. Kikuchi's disease: a case report and literature review. An Med Interna. 2003;20:247-50.



Onciu M, Medeiros LJ. Kikuchi-Fujimoto lymphadenitis. Adv Anat Pathol. 2003;10:204-11.



Payne JH, et al. Kikuchi-Fujimoto disease: a rare but important cause of lymphadenopathy. Acta Pediatr. 2003;92:162-4.



Thongsuksai P, et al. Histiocytic necrotizing lymphadenitis (Kikuchi's disease): clinico-pathologic characteristics of 23 cases and literature review. J Med Assoc Thai. 1999;82:812-18.



Kosch M, et al. [Kikuchi-Fujimoto disease: the differential diagnosis of cervical lymphadenitis with recurrent attacks of fever]. Dtsch Med Wochenschr. 1999;124:213-16.



Sato Y, et al. Histiocytic necrotizing lymphadenitis (Kikuchi's disease) with aseptic meningitis. J Neurol Sci. 1999;163:187-91.



JOURNAL ARTICLES

Lin HC. Kikuchi's disease: a review and analysis of 61 cases. Otolaryngol Head Neck Surg. 2003;128:650-3.



Chen YH, et al. Kikuchi disease in systemic lupus erythematosus: clinical features and literature review. Chung Hua min Kuo Wei Sheng Wu Chi Mien I Hsueh Tsa Chih. 1998;31:187-92.



Diera ML, et al. Kikuchi's disease with multisystemic involvement and adverse reaction to drugs. Pediatrics. 1999;104:e24



Cousin F, et al. Kikuchi disease associated with still disease. Int J Dermatol. 1999;38:464-67.



Iguchi H, et al. Apoptic cell death in Kikuchi's disease: a TEM study. Acta Otolaryngol Suppl (Stockh). 1998;112:898-900.

Resources

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Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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