55 Kenosia Avenue
Danbury, CT 06810
Phone: 203.744.0100
Toll Free: 1.800.999.6673
http://rarediseases.org

Kikuchi's Disease

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Copyright 1992, 2000, 2004, 2006

Synonyms of Kikuchi's Disease

Disorder Subdivisions

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.

Related Disorders

Symptoms of the following disorders can be similar to those of Kikuchi's disease. Comparisons may be useful for a differential diagnosis:

Burkitt's lymphoma is a cancer of the lymphatic system that affects the lymph nodes as well as other areas of the body. Tumors may occur in the kidneys, sex glands, jaw, bone marrow, or central nervous system as well as the lymph nodes. Burkitt's lymphoma may be infectious. This disorder occurs often in children living in Central Africa, and is associated with the Epstein-Barr virus.

Hodgkin's disease is a form of lymphatic cancer featuring tumorous lymph nodes. Fever, night sweats and weight loss may occur along with swollen lymph nodes. The diagnosis of Hodgkin's disease is confirmed if expert pathological examination shows the presence of so-called Reed-Sternberg cells. The exact cause of Hodgkin's disease is not known. (For more information on this disorder choose "Hodgkin" as your search term in the Rare Disease Database.)

Malignant lymphoma describes a malignant tumor of the lymphoid tissue that is cancerous. Within the lymph system a type of white blood cell (lymphocyte) circulates. Although the structure of these white blood cells may vary with the type of lymphoma present, the effects are usually similar. The appearance of large lymph nodes in the neck is usually followed by fever, weakness, weight loss and anemia. When there is widespread involvement of the lymphoid tissue, the spleen and liver may also enlarge.

The following conditions have been associated with Kikuchi disease in some patients. They are not necessary for a differential diagnosis:

Pancytopenia - a reduction in the number of red and white blood cells and platelets in the circulating blood.

Splenomegaly - an abnormal enlargement of the spleen.

Still's Disease - also called juvenile rheumatoid arthritis or rheumatic arthritis, is a form of arthritis that usually, but not exclusively, affects the larger joints of children. In addition to high intermittent fever and rash, patients present with inflammation of several thin sheets of tissue that line certain areas of the body (serous membranes). Consequently, the lymph nodes (lymphadenopathy) are affected. (For more information on this disorder, please choose "Rheumatic Arthritis" as your search term in the Rare Disease Database.)

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

Organizations related to Kikuchi's Disease

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.

Report last updated: 2008/03/31 00:00:00 GMT+0