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  3. Kikuchi disease
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Kikuchi disease


Title


Other Names:
Kikuchi necrotizing lymphadenitis; Nosocomial Kikuchi's disease; Kikuchi's disease; Kikuchi necrotizing lymphadenitis; Nosocomial Kikuchi's disease; Kikuchi's disease; Kikuchi-Fujimoto's disease; Histiocytic necrotising lymphadenitis; Histiocytic necrotizing lymphadenitis; Kikuchi-Fujimoto disease See More
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Summary Summary


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Kikuchi disease is a benign (non-cancerous) condition of the lymph nodes. The main symptoms include swollen lymph nodes in the neck, mild fever, and night sweats. Less common symptoms include weight loss, nausea, vomiting, and sore throat. While the exact cause of this condition is unknown, infectious and autoimmune causes have been suggested. Kikuchi disease usually gets better (resolves) on its own within one to four months (although it may take up to a year), with or without treatment. However, treatments are available to relieve some of the associated signs and symptoms.[1][2]
Last updated: 9/14/2016

Symptoms Symptoms


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Kikuchi disease is a benign disease of the lymph nodes. The main sign of Kikuchi disease is usually swollen lymph nodes in the neck, which tend to develop suddenly. Other signs and symptoms may include:[1][2]
  • Fever
  • Headache
  • Nausea and vomiting
  • Fatigue
  • Joint pain
  • Night sweats
  • Rash
  • Abdominal pain
Last updated: 9/14/2016

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 45 |
Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Cervical lymphadenopathy
Swollen lymph nodes in the neck
0025289
30%-79% of people have these symptoms
Alopecia
Hair loss
0001596
Anorexia 0002039
Chills 0025143
Cutaneous photosensitivity
Photosensitive skin
Photosensitive skin rashes
Photosensitivity
Sensitivity to sunlight
Skin photosensitivity
Sun sensitivity
[ more ]
0000992
Erythema 0010783
Fatigue
Tired
Tiredness
[ more ]
0012378
Increased lactate dehydrogenase activity 0025435
Leukopenia
Decreased blood leukocyte number
Low white blood cell count
[ more ]
0001882
Low-grade fever
Mild fever
0011134
Malar rash 0025300
Night sweats 0030166
Oral ulcer
Mouth ulcer
0000155
Palpebral edema
Fullness of eyelids
Puffy eyelids
Puffy lids
Swelling of eyelids
[ more ]
0100540
Pruritus
Itching
Itchy skin
Skin itching
[ more ]
0000989
Skin nodule
Growth of abnormal tissue on or under the skin
0200036
Vasculitis in the skin 0200029
Weight loss 0001824
5%-29% of people have these symptoms
Abnormal blistering of the skin
Blistering, generalized
Blisters
[ more ]
0008066
Abnormality of the gastrointestinal tract 0011024
Abnormality of the neck 0000464
Anemia
Low number of red blood cells or hemoglobin
0001903
Antinuclear antibody positivity 0003493
Arthralgia
Joint pain
0002829
Elevated C-reactive protein level 0011227
Elevated erythrocyte sedimentation rate
High ESR
0003565
Elevated hepatic transaminase
High liver enzymes
0002910
Enlargement of parotid gland 0011801
Erythematous macule 0025475
Interstitial pulmonary abnormality 0006530
Lymphocytosis
High lymphocyte count
0100827
Meningitis 0001287
Myalgia
Muscle ache
Muscle pain
[ more ]
0003326
Neutropenia
Low blood neutrophil count
Low neutrophil count
[ more ]
0001875
Papule 0200034
Skin erosion 0200041
Skin plaque 0200035
Splenomegaly
Increased spleen size
0001744
Thrombocytopenia
Low platelet count
0001873
1%-4% of people have these symptoms
Ataxia 0001251
Generalized lymphadenopathy
Generalized swelling of lymph nodes
Swollen lymph nodes affecting all regions of the body
[ more ]
0008940
Hepatomegaly
Enlarged liver
0002240
Myocarditis 0012819
Pleural effusion
Fluid around lungs
0002202
Pustule
Pimple
0200039
Showing of 45 |
Do you have more information about symptoms of this disease? We want to hear from you.
Last updated: 2/1/2019
Do you have updated information on this disease? We want to hear from you.

Cause Cause


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The cause of Kikuchi disease is not known. However, scientists have proposed two possible explanations for the disease. Some believe that it may be due to a virus or other infectious agent. Features that support this theory include Kikuchi disease's frequent association with symptoms similar to those of certain viral infections and the condition's spontaneous resolution (goes away on its own) with or without specific treatment.[1][2]

Others believe that it may be an autoimmune condition. According to this theory, Kikuchi disease occurs when one or more agents (for example, a virus) trigger a temporary immune response.[1][2]
Last updated: 9/14/2016

Inheritance Inheritance


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Kikuchi disease is not thought to be inherited. Most cases occur sporadically in people with no family history of the condition.[2] Recently, a case study reporting two familial cases of Kikuchi disease was published in the literature, raising the hypothesis of genetic factors or perhaps a predisposing genetic background.[3]
Last updated: 3/18/2016

Diagnosis Diagnosis


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A diagnosis of Kikuchi disease is often suspected based on the presence of certain signs and symptoms. The only test available to confirm the diagnosis is a lymph node biopsy. However, other types of laboratory tests and imaging studies may be recommended to support the diagnosis and/or rule out other conditions that cause similar features.[1][2]

Medscape Reference, a resource for medical professionals, offers more specific information about the diagnosis of Kikuchi disease. You may be asked to register to view the information but registration is free.
Last updated: 9/14/2016

Treatment Treatment


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Kikuchi disease generally goes away (resolves) on its own within one to four months, with or without intervention. However, treatments are available to relieve some of the associated signs and symptoms. For example, nonsteroidal anti-inflammatory drugs (NSAIDs) may be used to ease lymph node tenderness and fever. The use of corticosteroids has been recommended in severe cases.[1][2] Hydroxychloroquine (an immunosuppressant), alone or in combination with other treatments, has also been successfully used.[4]

Since there have been a few case reports of systemic lupus erythematosus (SLE or lupus) developing several years after recovery from Kikuchi disease, several medical researchers recommend long term follow-up to check for signs and symptoms of SLE.[2][5]

Medscape Reference, a resource for medical professionals, offers more specific information regarding the treatment and management of Kikuchi disease. You may be asked to register to view the information, but registration is free.
Last updated: 4/16/2018

Prognosis Prognosis


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The long-term outlook (prognosis) of Kikuchi disease is generally good. Lymph nodes usually return to normal size within 1-6 months after onset, although it may take longer. About 3% to 4% of people may develop Kikuchi disease again.[1][2]

Kikuchi disease has been found to be associated with several autoimmune diseases, especially systemic lupus erythematous (SLE or lupus). Cases include those in which SLE began before Kikuchi disease, at the same time as Kikuchi disease, and in a few cases, several years after Kikuchi disease. Therefore some medical researchers recommend long term follow up to make check for the SLE.[1][5] 

SLE is a chronic autoimmune disorder that may affect the skin, joints, kidneys, and other organs. Symptoms vary from person to person although almost all people with SLE have joint pain and swelling. Some develop arthritis. Other symptoms may include chest pain when taking a deep breath; general discomfort or ill feeling; and/or mouth sores.[6]
Last updated: 9/14/2016

Research Research


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Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Kikuchi disease. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Learn More Learn More


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These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Kikuchi disease. Click on the link to view a sample search on this topic.

News & Events News & Events


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News

  • NIH Hosts Rare Disease Day Event, Twitter Chat
    January 24, 2019

GARD Answers GARD Answers


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Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • Can this affect young men also? My son who is 34 always has swollen lymph nodes/glands and has mixed connective tissue disease (MCTD). He feels very poor most of the time but can't seem to get a diagnosis. See answer

  • I was diagnosed with Kikuchi's disease a number of years ago. I had a lump on the side of my neck that kept on getting bigger. An ear nose and throat specialist did surgery to remove it. Biopsy confirmed that it was Kikuchi's disease. The ear nose and throat specialist informed me that I would have these lumps throughout my body for the rest of my life. Is this true? According to what I have been reading online it is supposed to go away after just a few months? I have a new lump on the other side of my neck now that has been getting bigger and it hurts. I had an MRI done and they said that it is only 1 cm wide on the inside but on the outside it was 5cm by 6cm. Do you think that I should have this removed? See answer

  • I am suffering from Kikuchi disease. What kind of treatment should I undergo and is taking painkillers the right treatment? See answer


Have a question? Contact a GARD Information Specialist.

References References


  1. Michael J Richards, MD, FRACP. Kikuchi disease. UpToDate. May 2015; http://www.uptodate.com/contents/kikuchi-disease.
  2. John Boone. Kikuchi disease. Medscape. February 2016; http://emedicine.medscape.com/article/210752-overview.
  3. A.-L. Lecapitainea, J. Chevaliera, B. Juberthieb, M.-A. Bouldouyrea, H. Gros. Kikuchi-Fujimoto's disease or histiocytic necrotizing lymphadenitis: A report of two familial cases [article in French]. La Revue de Médecine Interne. 2/19/2016; http://www.ncbi.nlm.nih.gov/pubmed/26907374.
  4. Dumas G, Prendki V, Haroche J et al. Kikuchi-Fujimoto disease: retrospective study of 91 cases and review of the literature. Medicine (Baltimore). November, 2014; 93(24):372-382. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4602439/.
  5. Baenas DF, Diehl FA, Haye Salinas MJ, Riva V, Diller A, and Lemos PA. Kikuchi–Fujimoto disease and systemic lupus erythematosus. International Medical Case Reports Journal. June 2016; 9:163-167. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3644467/.
  6. Borigini MJ. Systemic lupus erythematosus. MedlinePlus. January 2016; http://www.nlm.nih.gov/medlineplus/ency/article/000435.htm.
Do you know of a review article? We want to hear from you.
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rare disease research!
You can help advance rare disease research!
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