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Disease Profile
Kikuchi disease
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.
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.
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.
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.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
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.
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.
Not applicable
Other names (AKA)
Kikuchi necrotizing lymphadenitis; Nosocomial Kikuchi's disease; Kikuchi's disease;
Summary
Kikuchi disease is a benign (non-cancerous) condition of the
Symptoms
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 | |
Low number of red blood cells or hemoglobin
|
0001903 | |
Antinuclear |
0003493 | |
Arthralgia |
Joint pain
|
0002829 |
Elevated C-reactive |
0011227 | |
Elevated |
High ESR
|
0003565 |
Elevated hepatic transaminase |
High liver enzymes
|
0002910 |
Enlargement of parotid gland | 0011801 | |
Erythematous macule | 0025475 | |
Lymphocytosis |
High lymphocyte count
|
0100827 |
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 |
Low platelet count
|
0001873 | |
1%-4% of people have these symptoms | ||
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
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
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
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
- Michael J Richards, MD, FRACP. Kikuchi disease. UpToDate. May 2015; https://www.uptodate.com/contents/kikuchi-disease.
- John Boone. Kikuchi disease. Medscape. February 2016; https://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; https://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; https://www.nlm.nih.gov/medlineplus/ency/article/000435.htm.
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