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What

The Kikuchi syndrome is a rare disorder that results in the inflammation and swelling of lymph nodes. Glands in the neck are most commonly affected and children most commonly have symptoms that resolve on their own.

Who

Kikuchi syndrome is rare in the United States and most commonly affects individuals of Asian descent, especially Koreans. Females are affected more than males in a 4 to 1 ratio. There is also a wide age range of children affected beginning as early as 10 months.

Signs and Symptoms

Children with Kikuchi syndrome usually have the following symptoms:

• High fevers
• Muscle and joint pain
• Neck gland swelling with pain and tenderness
• Skin rash that appears as red bumps on the body (about 33% of children)
• Nausea, vomiting, and sweats occasionally.
• Enlargement of the liver and spleen
• Visual changes These symtpoms usually begin fairly suddenly and resolve by themselves over about 6 months.

Possible Causes

The cause of this rare disorder is still unknown. The disorder is believed to be a type of autoimmune disease. It is possible that an infection may trigger the disease, but what type of infection is not known. The disorder has also been described in association with other autoimmune diseases, such as Sjögren’s syndrome and lupus.

Diagnosis

There are no specific blood tests that can be used to diagnose Kikuchi syndrome. Affected individuals will generally display signs of inflammation on blood tests that include: elevated sedimentation rate (ESR), white blood cells with an unusual appearance (atypical lymphocytes), and either a high or low white blood cell count. Children usually have low white blood cell counts for an unknown reason. Your doctor may order a CT scan (computerized tomography) to visualize enlarged lymph nodes throughout the body. To make a definitive diagnosis a biopsy of the affected gland or skin may be performed and studied under a microscope.

Treatment

Children with Kikuchi syndrome are treated with medicines to reduce the fever and relieve symptoms, and the problem typically resolves over about 6 months. Once the diagnosis is made by biopsy and/or blood cell analysis, parents can be reassured. Contact sports should be avoided in children with enlarged spleens or livers because of the risk of organ injury.

Prognosis

Kikuchi syndrome is a self-limited disease that reoccurs in approximately 3% of patients. Most children recover without any permanent disability.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Health on the net foundation
Central website for the Internet savvy parent.

National organization of rare disorders (NORD)
Disease Information of national organizations that can help.

The Doctor’s doctor
More technical information but useful nonetheless.

Diseases database.com
More links to information on this rare disease.

MedHelp International
search “lymphadenitis” General information on lymph node swelling for parents.

Google Search for Kikuchi Syndrome

References and Sources

EMedicine article: Noah Scheinfeld http://www.emedicine.com/derm/topic931.html