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

Summary Summary

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

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]

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.

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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		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		0002202
Pustule	Pimple	0200039

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Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 10/1/2018

Do you have updated information on this disease? We want to hear from you.

Cause Cause

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

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

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

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

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

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

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

News

NCATS Rare Diseases Are Not Rare! Challenge
October 9, 2018
The NIH Undiagnosed Diseases Network Expands
September 26, 2018

GARD Answers GARD Answers

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

Michael J Richards, MD, FRACP. Kikuchi disease. UpToDate. May 2015; http://www.uptodate.com/contents/kikuchi-disease.
John Boone. Kikuchi disease. Medscape. February 2016; http://emedicine.medscape.com/article/210752-overview.
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.
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/.
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/.
Borigini MJ. Systemic lupus erythematosus. MedlinePlus. January 2016; http://www.nlm.nih.gov/medlineplus/ency/article/000435.htm.