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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.