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Disease Profile

Kikuchi disease

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

Adolescent

ICD-10

I88.1

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Kikuchi necrotizing lymphadenitis; Nosocomial Kikuchi's disease; Kikuchi's disease;

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]

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]

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.

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 level
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
Abnormal pulmonary Interstitial morphology
Abnormality in area between air sacs in lung
0006530
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
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
Inflammation of heart muscle
0012819
Pleural effusion
Fluid around lungs
0002202
Pustule
Pimple
0200039

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]

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.

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.

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.

References

  1. Michael J Richards, MD, FRACP. Kikuchi disease. UpToDate. May 2015; https://www.uptodate.com/contents/kikuchi-disease.
  2. John Boone. Kikuchi disease. Medscape. February 2016; https://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; https://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; https://www.nlm.nih.gov/medlineplus/ency/article/000435.htm.

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