$Unique_ID{BRK03903} $Pretitle{} $Title{Kikuchi's Disease} $Subject{Kikuchi's Disease Histiocytic Necrotizing Lymphadenitis HNL Kikuchi-Fujimoto Disease Kikuchi's Histiocytic Necrotizing Lymphadenitis Necrotizing Lymphadenitis Burkitt's Lymphoma Hodgkin Disease Malignant Lymphoma } $Volume{} $Log{} Copyright (C) 1992 National Organization for Rare Disorders, Inc. 861: Kikuchi's Disease ** IMPORTANT ** It is possible that the main title of the article (Kikuchi's Disease) is not the name you expected. Please check the SYNONYMS listing to find the alternate name and disorder subdivisions covered by this article. Synonyms Histiocytic Necrotizing Lymphadenitis HNL Kikuchi-Fujimoto Disease Kikuchi's Histiocytic Necrotizing Lymphadenitis Necrotizing Lymphadenitis Information on the following diseases can be found in the Related Disorders section of this report: Burkitt's Lymphoma Hodgkin Disease Malignant Lymphoma General Discussion ** REMINDER ** The Information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Kikuchi's Disease is a rare noncancerous disorder in which there are lesions that typically affect the lymph nodes in the neck of young adults. This disorder is often mistaken for malignant Lymphoma because the symptoms are very similar. The lesions, or tissue abnormalities in this disorder cause the lymph nodes to become enlarged, inflamed and painful. The exact cause of Kikuchi's Disease is not known. Symptoms Kikuchi's Disease is a rare nonmalignant disorder that affects the lymph nodes. The lymph nodes are small oval structures that filters lymph fluid that draws from organs and tissues, fight infection and form white blood cells and blood plasma cells. In Kikuchi's Disease lymph nodes on the side of the neck and near the salivary glands are affected. The lymph nodes may be swollen, hard, painful and tender. The patient may have a fever and pain in the area of the affected lymph nodes. The abnormal tissue growths and inflammation in Kikuchi's Disease normally clear up within a few weeks or months without treatment. Causes The exact cause of Kikuchi's Disease is not known. Many researchers suspect that a virus may cause this disorder. Affected Population Kikuchi's Disease is an extremely rare disease. There have been less than 50 cases reported in the medical literature. This disorder particularly affects young adults. Kikuchi's Disease was originally identified in Japan in 1972. Kikuchi's Disease may be more prevalent than has been previously recorded due to the fact that swollen glands are fairly common and ignored by many people. Since this disorder can only be determined by a biopsy of the tissue in the affected lymph nodes, it may be overlooked by many unsuspecting patients. 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 cancer of the lymphatic system, especially the lymph nodes. Tumors occur in the lymph nodes. Fever, night sweats and weight loss may occur along with swollen lymph nodes. Examination of the affected lymph node tissue by a pathologist shows the presence of a type of cell 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 is a tumor of the lymphoid tissue that is cancerous. Lymphoid tissue contains a type of white blood cell (lymphocyte) in its spaces. The cell structure varies in the various types of lymphomas, but the effects of these tumors 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's 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 affects the larger joints of children. It may also occur in adulthood. The patient may have a high intermittent fever, rash, inflammation of several thin sheets of tissue that line certain areas of the body (serous membranes), a disease process that affects the lymph nodes (Lymphadenopathy), enlargement of the liver and spleen, an abnormal increase in the number of circulating white blood cells (leukocytosis), and a decrease in red cells in the blood (anemia). This disorder is often mistaken for leukemia or an infection. (For more information on this disorder, please choose "Rheumatic Arthritis" as your search term in the Rare Disease Database.) Therapies: Standard Treatment of Kikuchi's Disease is symptomatic and supportive. Usually the disorder resolves itself spontaneously within a few weeks or months. 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 if the patient has the disease. Therapies: Investigational This disease entry is based upon medical information available through April 1992. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Kikuchi's Disease, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Institute of Allergy and Infectious Diseases 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5717 References KIKUCHI-FUJIMOTO DISEASE MIMICKING MALIGNANT LYMPHOMA: G.A. Chamulak, et al., Am J Surg Pathol (June 1990, issue 14(6)). Pp. 514-23. NECROTIZING LYMPHADENITIS (KIKUCHI'S DISEASE). REPORT OF FOUR CASES OF AN UNUSUAL PSEUDOLYMPHOMATOUS LESION AND IMMUNOLOGIC MARKER STUDIES: P.D. Unger, et al.; Arch Pathol Lab Med (November 1987, issue 111(11)). Pp. 1031-4. CUTANEOUS MANIFESTATIONS OF KIKUCHI'S HISTIOCYTIC NECROTIZING LYMPHADENITIS: T.T. Kuo; Am J Surg Pathol (September 1990, issue 14(9)). Pp. 872-6. KIKUCHI-FUJIMOTO DISEASE MIMICKING MALIGNANT LYMPHOMA: G.A. Chamulak, et al.; Am J Surg Pathol (June 1990, issue 14(6)). Pp. 514-23. NECROTISING LYMPHADENITIS WITHOUT GRANULOCYTIC INFILTRATION (KIKUCHI'S DISEASE): M.H. Ali, et al.; J Clin Pathol (November 1985, issue 38(11)). Pp. 1252-7.