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Richard thought he had a case of jock itch that just wouldn't go away, but he actually had a rare form of cancer of the sweat glands known as extramammary Paget disease. Here's what men and women need to know about this potentially deadly form of cancer.

Richard had a job that required him to travel from his home near Baltimore, Maryland to his employer's office in London once a month. On one of his trips in 2015, he received an urgent message from home just before boarding his plane.

The caller was his doctor with test results that had come back much sooner than expected. Richard had to take notes on the back of his boarding pass about some condition he had never heard of called extramammary Paget disease. On the six-hour flight home the plane's intermittent Internet connection did not give him many opportunities to find out what he was facing when he returned home.

Jock Itch Gone Very, Very Bad

Three years earlier Richard had seen his doctor for a routine checkup and mentioned only in passing that he had a case of jock itch that just wouldn't go away. Several months before that, Richard had noticed a painful, purple pimple on his scrotum. He assumed it was an ingrown hair left behind by shaving. The pimple eventually disappeared, but was replaced by small scaly patch of red skin about the size of a dime (18 mm). He showed the red patch to his primary care doctor, who diagnosed it as a tineal, athlete's foot-like fungal infection. The doctor told him to get an over-the-counter cream.

The over-the-counter product didn't work, and the spot on his balls was becoming increasingly itchy. He went back to the doctor, who then prescribed a stronger anti-fungal product. That medication didn't work, either, so Richard eventually got an appointment with a dermatologist. The dermatologist concluded that the spot had to be contact dermatitis, probably an allergic reaction to the elastic in his briefs. He told Richard to switch to boxers and gave him a steroid cream. 

A full year passed and the itch only got worse. Richard changed brands of detergent and stopped using fabric softener. He took antihistamines. He found a brand of hypoallergenic underwear. Nothing worked, until the dermatologist finally did a biopsy. That was when Richard learned he had a relatively rare condition called extramammary Paget disease.

What Is Extramammary Paget Disease? 

Extramammary Paget disease is a form of cancer that develops in apocrine sweat glands, the sweat glands that are found under the armpits, around the nipples, around the genitals, and in the ears. The cancer can spread to nearby tissues. The colon, urethra, and bladder can develop tumors in both men and women, the prostate in men, and the cervix and uterus in women. The condition is three to five times more common in women than in men.

Usually the only symptom of extramammary Paget disease is intense itching. The affected skin may bleed from scratching, or from the pressure of a tumor. Sometimes the condition can be treated with an immune response modifier called imiquimod (INN), but usually treatment requires surgery followed up with chemotherapy delivered in the form of creams. Even when the cancer is removed by surgery, it comes back in about 30 percent of cases, two to ten years later. More people survive extramammary Paget disease than not. A diagnosis of the condition is not necessarily a death sentence, but it can be fatal if not treated or without followup care.

What Do I Need to Know If I Have Been Diagnosed with Extramammary Paget Disease?

The first thing every patient diagnosed with extramammary Paget disease needs to verify is the diagnosis. There is more than one form of "Paget disease."

  • Paget disease is a bone deformity first described by English surgeon James Paget in 1877. In this condition, the bones first become excessively weak and then become hyperactive in their repair. The result is large, misshapen, "woven" bones that break easily. Paget disease can affect just one bone, but it is more commonly multifocal, affecting bones in different places in the body. It affects one to three million people in the United States alone, most commonly people over age 65, and occurs slightly more often in men than in women. Different races get Paget disease at approximately the same rate. The condition causes bone pain, arthritis, bone deformities, and neurological issues caused by bone pressing against brain or nerves.

  • Mammary Paget disease is breast disease also described by James Paget, in 1874. It presents itself as itchy redness of the areola and nipple. It occurs almost exclusively in women, and when it occurs in women, it almost always signals breast cancer. About one in 250 women will develop the condition at some time in their lives. Cases have been diagnosed in women as young as 24 and as old as 84. Men who develop the condition may also have breast cancer; they usually have been treated for prostate cancer with estrogen.
  • Extramammary Paget disease was first described by Radcliffe Crocker in 1889, who noted that the physical symptoms and laboratory exams of extramammary Paget disease (Paget disease that causes skin irritation other than on the breast) are identical to those for mammary Paget disease. This condition is much rarer than the other two, affected only a few hundred to a few thousand people in the world.

The term "Paget's disease" is used interchangeably with "Paget disease."

Extramammary Paget disease may cause annoying symptoms for 10 or even 20 years without infiltrating surrounding organs with rumor cells. However, once it transforms itself into cancer, extensive surgery may be needed to keep the cancer from spreading. Loss of the vulva and cervix in women or the testicles, penis, and perianal areas in men are possible consequences of delaying treatment, and aren't the worst possibilities for the disease. Even when extramammary Paget disease is treated, it can come back, and the same consequences of neglecting care can occur later rather than sooner.

The moral of Richard's story to men and women alike is that skin itches that seem to be fungal infections, yeast infections, eczema, or contact dermatitis that just won't go away may not be what they appear. If you have even a small itch on breast or genitals that persists for more than six months, you need to persist with your doctors and with a dermatologist until you get the treatment you need.

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