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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.

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.