Rare Disease Sometimes Misdiagnosed As Lymphoma

Dear Dr. Roach: What can you tell me about Kikuchi-Fujimoto disease? My 13-year-old granddaughter has been diagnosed with this disease, and we have very little information.

In fact, the EENT who diagnosed her said it was the only case he has ever seen.

They did a biopsy of the lymph nodes and are treating her with antibiotics and steroids.

Should we be looking for a research hospital and a doctor who has treated this disease before? Can it be cured, or only go into remission? What should we be concerned about?

Any information or recommendations you can provide would be greatly appreciated. — S.K.

Answer: Kikuchi-Fujimoto disease is a rare disease (I have never seen a case) of unknown cause, first reported in Japan but present in every ethnic group.

It is thought to be caused by a virus, but that has never been proven.

The major symptoms are tender, enlarged lymph nodes, usually with fever and sweats at nighttime. Occasionally there are other symptoms, such as rash, weight loss, nausea, vomiting and sore throat.

Fortunately, in most people, the disease goes away as mysteriously as it came, after one to four months. No treatment is consistently effective, although severe cases have been treated with steroids, immune globulin or other medications. Ten percent to 20 percent of cases will have a relapse.

The expertise that is needed is in the pathologist who reviews the biopsy.

Cases have been misinterpreted as lymphoma, a blood cancer.

Dear Dr. Roach: My granddaughter is 11 and has cystic fibrosis. She is doing very well.

Her hands and feet sweat profusely, to the point of wetting her school papers.

She is very embarrassed by it. What do you recommend? — E.J.

Answer: Cystic fibrosis is caused by an abnormality in the CFTR gene, and this affects many of the body’s functions, especially in secretion of fluids.

The pancreas can be affected due to thickened and abnormal secretions, leading to pancreatic disease. The lungs may be obstructed due to the thick secretions, predisposing the person with CF to lung infections.

The sweat glands also are abnormal, and hyperhidrosis (excessive sweating) is prevalent. Regular antiperspirant can be applied to the hands.

There are prescription antiperspirants if the over-the-counter brands aren’t effective.

They are best applied when sweating is at a minimum, such as at bedtime.

Dear Dr. Roach: I have a desmoid tumor of the neck and head. I was operated on in 2008 and again in 2012. I realize that the reoccurrence rate is high for this type of nonmalignant tumor.

When the tumor comes back again, my physician told me that I would need reconstructive surgery. I am electing not to have radiation.

Could this tumor spread to the brain? — K.D.

Answer: A desmoid tumor is a locally aggressive tumor. It isn’t a cancer, so it can’t spread by blood or lymphatic tissues, the way cancers can.

However, in this case, the term “benign” can be misleading, since they are so difficult to get rid of. They are most common in the abdomen, trunk or extremities, but can occur in virtually any body area.

Radiation does decrease risk of recurrence.

Some centers are using medications such as tamoxifen or even chemotherapy in people with recurrent disease, or in those not amenable to surgery.

Dr. Roach regrets that he is unable to answer individual letters, but will incorporate them in the column whenever possible. Readers may email questions to ToYourGoodHealth@med.cornell.edu or request an order form of available health newsletters at P.O. Box 536475, Orlando, FL 32853-6475. Health newsletters may be ordered from www.rbmamall.com.