What is Kikuchi Disease?

Histiocytic necrotizing lymphadenitis
Nephron/Wikipedia Commons/Creative Commons

Kikuchi disease, also called histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease, is a disease that affects the lymph nodes. Learn more about who is at risk, plus the symptoms, diagnosis and treatments for this rare, not life threatening disease.

What is Kikuchi Disease?

Kikuchi disease causes lymph node inflammation. The exact cause of the disease is not yet known, although some researchers have suggested it is an infection or autoimmune disorder.

The most widely accepted theory by experts is that Kikuchi disease is the result of one or more unidentified agents triggering a self-limited autoimmune process. These agents are thought to include infections, chemical, plastic and neoplastic (abnormal tissue growth) agents.

Who is at Risk?

Kikuchi disease was first described in Japan in 1972, but has since been reported throughout the world in all races. It was previously thought that more women than men were affected by Kikuchi disease, however more recent evidence suggests that it is not more likely to affect one sex more than another. Kikuchi disease occurs in a wide age range, but generally affects young adults ages 20 to 30.

Symptoms of Kikuchi Disease

Kikuchi disease usually takes the form of lymph node inflammation. In 80 percent of people with the disease, the lymph nodes on one or both sides of the neck are usually affected. Most of the time, these are the only lymph nodes affected.

The lymph nodes are painless, hard and around 2-3 cm in diameter. About half of people with Kikuchi disease develop a fever and flu-like symptoms. A red rash may appear in up to 30 percent of individuals.

Diagnosing Kikuchi Disease

An ultrasound, CT scan or MRI can confirm the presence of enlarged lymph nodes but cannot confirm the diagnosis.

Taking out a sample of a lymph node also cannot confirm the diagnosis. Because of its symptoms and because it is so difficult to diagnose, Kikuchi disease is often mistaken for lymphoma or systemic lupus erythematosus. The only way to know for sure if your symptoms are from Kikuchi disease is for your doctor to remove a lymph node and examine the tissues in it. Luckily, unlike lymphoma and lupus, Kikuchi disease is not life threatening or chronic in nature.

Treatment Options

Treatment for Kikuchi disease consists of relieving any fever, flu symptoms or lymph node tenderness. Nonsteroidal antinflammatory drugs (NSAIDs), such as ibuprofen, can help with these symptoms. Kikuchi disease will usually clear up on its own within one to six months.

Take a look at these resources for more information about lymph node diseases and disorders:

Are Enlarged Lymph Nodes Always a Sign of Lymphoma?

Blood and Lymph Cancer Basics

What Are Lymph Nodes?

Sources:

Boone, J. L. (2004). Kikuchi disease. eMedicine.

Chaitanya, B. N., & Sindura, C. S. (2010). Kikuchi's disease. J Oral Maxillofac Pathol, 14(1), 6-9.

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