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Genetic and Rare Diseases Information Center (GARD)

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Kimura disease

Other Names for this Disease
  • Angiolymphoid hyperplasia with eosinophilia
  • Eosinophilic granuloma of soft tissue
  • Eosinophilic hyperplastic lymphogranuloma
  • Eosinophilic lymphofollicular granuloma
  • Eosinophilic lymphofolliculosis
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Your Question

I have Kimura disease on my ear lobe. My right ear lobe has a swollen lump which was removed 4 years ago, but it returned one month later. Last year, I treated it with prednisone for 2 weeks which caused the lump to shrink back to normal.  When the dosage of prednisone was halved, the lump started to come back again. I am currently taking the higher dose of prednisone once again.  I want to know if there is any other medication for this disease.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Kimura disease?

Kimura disease is a rare, benign, chronic disorder that causes inflammation of tissue (nodules) under the skin of the head or neck.  These nodules tend to recur despite treatment. The cause of this condition is unknown, but may be due to an immune response. [1]
Last updated: 1/20/2011

How might Kimura disease be treated?

For individuals with symptoms caused by Kimura disease, surgery to remove the nodules is the treatment of choice; however, the nodules often reappear after surgery. [2]

Steroids (such as prednisone), taken by mouth or via an injection in the skin, can shrink the nodules but rarely result in a cure. Other, less common, treatments include oral pentoxifylline, medication that supresses the immune system (such as cyclosporine), radiotherapy, and a combination of all trans-retinoic acid and prednisone. [2] [1]

It is important to consult with your healthcare provider before taking any medication.
Last updated: 5/26/2011