Rare Diseases Types Symptoms and Treatment of Kikuchi Disease By Mary Kugler, RN Mary Kugler, RN Mary Kugler, RN, is a pediatric nurse whose specialty is caring for children with long-term or severe medical problems. Learn about our editorial process Updated on February 13, 2023 Medically reviewed by Ronald Lubelchek, MD Medically reviewed by Ronald Lubelchek, MD LinkedIn Ronald Lubelchek, MD, is a board-certified physician and medical director in Chicago, Illinois who specializes in infectious diseases. Learn about our Medical Expert Board Fact checked by Angela Underwood Fact checked by Angela Underwood LinkedIn Angela Underwood's extensive local, state, and federal healthcare and environmental news coverage includes 911 first-responder compensation policy to the Ciba-Geigy water contamination case in Toms River, NJ. Her additional health-related coverage includes death and dying, skin care, and autism spectrum disorder. Learn about our editorial process Print Kikuchi disease, also called histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease, is a disease that affects the lymph nodes, causing lymph node inflammation. The exact cause of the disease is not yet known, although some researchers have suggested it is an infection or autoimmune disorder. The most widely accepted theory by experts is that Kikuchi disease is the result of one or more unidentified agents triggering a self-limited autoimmune process. These agents are thought to include infections, chemical, plastic, and neoplastic (abnormal tissue growth) agents. Nephron / Wikimedia Commons / CC BY-SA 3.0 Who's at Risk? Kikuchi disease was first described in Japan in 1972 but has since been reported throughout the world in all races. It was previously thought that more women than men were affected by Kikuchi disease; however, more recent evidence suggests that it is not more likely to affect one sex more than another. Kikuchi disease occurs in a wide age range, but generally affects young adults ages 20 to 30. Symptoms Kikuchi disease usually takes the form of lymph node inflammation. In 80 percent of people with the disease, the lymph nodes on one or both sides of the neck are affected. Most of the time, these are the only lymph nodes affected. About half of people with Kikuchi disease develop a fever and flu-like symptoms. A red rash may appear in up to 30 percent of individuals. The lymph nodes can be tender and painful around 2-3 cm in diameter. Diagnosis An ultrasound, CT scan, or MRI can confirm the presence of enlarged lymph nodes but cannot confirm the diagnosis.Because of its symptoms and because it is so difficult to diagnose, Kikuchi disease is often mistaken for lymphoma or systemic lupus erythematosus. The only way to know for sure if your symptoms are from Kikuchi disease is for your healthcare provider to remove a lymph node and examine the tissues in it. Luckily, unlike lymphoma and lupus, Kikuchi disease is not life-threatening or chronic in nature. Treatment Options Treatment for Kikuchi disease consists of relieving any fever, flu symptoms, or lymph node tenderness. Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen can help with these symptoms. Kikuchi disease will usually clear up on its own within one to four months. 10 Sources Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy. Perry AM, Choi SM. Kikuchi-fujimoto disease: a review. Archives of Pathology & Laboratory Medicine. 2018;142(11):1341-1346. doi: 10.5858/arpa.2018-0219-RA Susheelan V, Thambi R, Mathew S. Kikuchi′s disease: A study of 96 cases over a 12-year period. Saudi J Health Sci. 2016;5(3):134. doi: 10.4103/2278-0521.195818 Lamzaf L, Harmouche H, Maamar M, Adnaoui M, Aouni M, Tazi Mezalek Z. Kikuchi-Fujimoto disease: Report of 4 cases and review of the literature. European Annals of Otorhinolaryngology, Head and Neck Diseases. 2014;131(6):329-332. doi: 10.1016/j.anorl.2013.01.007 National Center for Advancing Transational Sciences. Kikuchi disease. Lelii M, Senatore L, Amodeo I, et al. Kikuchi-Fujimoto disease in children: two case reports and a review of the literature. Ital J Pediatr. 2018;44(1):83. doi: 10.1186/s13052-018-0522-9 Cui XW, Jenssen C, Saftoiu A, Ignee A, Dietrich CF. New ultrasound techniques for lymph node evaluation. World Journal of Gastroenterology. 2013;19(30):4850-4860. doi: 10.3748/wjg.v19.i30.4850 Găman M, Vlădăreanu AM, Dobrea C, et al. A challenging case of kikuchi-fujimoto disease associated with systemic lupus erythematosus and review of the literature. Case Reports in Hematology. 2018;2018:1-5. doi: 10.1155/2018/1791627 Al Manasra AR, Al-Domaidat H, Aideh MA, et al. Kikuchi–fujimoto disease in the eastern mediterranean zone. Sci Rep. 2022;12(1):2703. doi: 10.1038/s41598-022-06757-9 Honda F, Tsuboi H, Toko H, et al. Recurrent kikuchi-fujimoto disease successfully treated by the concomitant use of hydroxychloroquine and corticosteroids. Internal Medicine. 2017;56(24):3373-3377. doi: 10.2169/internalmedicine.9205-17 Deaver DM, Naghashpour M, Sokol L. Kikuchi-fujimoto disease in the united states: three case reports and review of the literature. Mediterranean Journal of Hematology and Infectious Diseases. 2014;6(1):e2014001-e2014001. doi: 10.4084/MJHID.2014.001 By Mary Kugler, RN Mary Kugler, RN, is a pediatric nurse whose specialty is caring for children with long-term or severe medical problems. See Our Editorial Process Meet Our Medical Expert Board Share Feedback Was this page helpful? Thanks for your feedback! 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