What are the other Names for this Condition? (Also known as/Synonyms)

• Eosinophilic Lymphogranuloma

What is Kimura Disease? (Definition/Background Information)

• Kimura Disease is a rare, benign and chronic inflammatory disorder, that primarily affects the lymph nodes and tissue under the skin in the head and neck region of the body. The mouth, armpits, limbs, groin, and torso may also be affected by this condition
• The disorder is most common in Asia. Males between the ages of 20 and 40 years are most likely to develop Kimura Disease
• The exact cause of the disorder is not known, although it is generally believed that a dysfunctional immune system may lead to Kimura Disease
• The disease manifests itself as small and painless swellings on skin that gradually increases in size. These lesions are observed microscopically after surgical excision for an accurate diagnosis
• Surgery is the mainstay of treatment for the condition. If left untreated, Kimura Disease may lead to complications such as nephrotic syndrome and eosinophilic panniculitis
• The prognosis of Kimura Disease is excellent with proper medical care

Who gets Kimura Disease? (Age and Sex Distribution)

• Kimura Disease is a rare inflammatory condition of the skin and lymph nodes. There has been a very limited number of cases of Kimura Disease reported worldwide
• People of Asian descent are most likely to develop this inflammatory condition. A few rare cases have been reported outside of Asia
• Young adult males between 20-40 years of ages are most prone to the condition
• Both genders are susceptible to the disorder, although males are up to 7 times more vulnerable to developing the condition when compared to females

What are the Risk Factors for Kimura Disease? (Predisposing Factors)

The risk factors of Kimura Disease include the following:

• Being of Asian descent
• Male gender
• Being 20 to 40 years old

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Kimura Disease? (Etiology)

The exact cause of Kimura Disease is not fully known. It is theorized that Kimura Disease may be caused by the following factors:

• An abnormal immune response that is possibly due to:
  • Allergic reactions
  • Infections
  • Autoimmune reactions
• This abnormal immune response occurs over a long period of time, due to prolonged stimulation of the immune system

What are the Signs and Symptoms of Kimura Disease?

The signs and symptoms of Kimura Disease may include: 

• One or many painless growths of abnormal tissue beneath the skin. These growths are typically located in the head and neck region, but may be also found at the following body sites:
  • Mouth
  • Armpits
  • Groin
  • Limbs
  • Torso
• Enlarged salivary glands and swollen lymph nodes
• An increased number of eosinophils in blood (or eosinophilia): Eosinophils are a type of white blood cells that the body generates for defense, in response to allergens, drugs, and parasites
• An increased number of IgE antibodies in blood. IgE antibodies are produced by the body in response to allergies
• Protein in the urine (proteinuria)
• Swelling in the lower extremities, if nephrotic syndrome develops
• Presence of nodules and joint pain, accompanied by fever and fatigue

How is Kimura Disease Diagnosed?

The diagnosis of Kimura Disease is made through the following tools:

• A complete physical examination and review of medical or travel history
• Assessment of symptoms
• Microscopic examination of surgically removed lesions for specific patterns and eosinophil infiltration
• Blood tests for eosinophil and IgE antibody counts
• Urine analysis for detection of protein in urine

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Kimura Disease?

Complications of Kimura Disease may include the following: 

• Eosinophilic panniculitis: It is a disorder characterized by eosinophil infiltration of fat below the skin (causing nodule formation)
• Nephrotic syndrome: It is a disorder that causes the body to excrete protein in urine. It may be due to damaged blood vessels in the kidneys that normally does not allow protein to be secreted. This syndrome may cause swelling in the lower extremities

How is Kimura Disease Treated?

The following are treatment options for Kimura Disease:

• Surgical removal of skin lesions
• Treatment with steroids, especially corticoids, if there is kidney involvement
• Radiation therapy
• Treatment with cytotoxic agents (drugs that kill cells)

How can Kimura Disease be Prevented?

There is no known prevention for Kimura Disease, as the cause is not fully-understood.

• Early detection and treatment is beneficial for increasing the probability of curing the condition
• Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as Kimura Disease
• Regular medical screening at periodic intervals with tests and physical examinations are strongly recommended

What is the Prognosis of Kimura Disease? (Outcomes/Resolutions)

The prognosis for Kimura Disease is good with proper treatment. The possible outcomes of Kimura Disease include the following:

• With adequate treatment, the signs and symptoms may be eliminated successfully
• In some cases, relapses can occur in those who have received treatment. With additional and symptomatic treatment, these relapses can be addressed as well
• If left untreated, the condition can lead to disorders such as nephrotic syndrome and eosinophilic panniculitis

Additional and Relevant Useful Information for Kimura Disease:

• Kimura Disease was first described in China by Kim and Szeto in 1937; then, later in 1948 by Kimura et al in Japan, giving the condition its name
• Angiolymphoid hyperplasia with eosinophilia (ALHE) was initially thought to be related to Kimura Disease; however, further studies have indicated that these two conditions are clinically and radiologically different