What is paget’s disease (pd)?
Paget’s disease (pd) is a rare skin cancer that can appears around the nipple, vulva or anus in women and in extremely rare cases around the penis, scrotum, groin and perianal region in men.
Since pd is so rare, it is often difficult to find a physician that is familiar with the disease. Many doctors have never heard of it and even fewer have seen it. Aggressive surgical intervention is often necessary although other treatments are also used. Sometimes pd can point to underlying malignancies.
Extramammary paget’s disease (pd) often appears as a scaly, itchy or red area within the nipple or genital region. This cancer is extremely rare, generally slow growing, and is often misdiagnosed as eczema. The prognosis and recovery from pd is often good, especially if it is found and treated early. However some cases of pd can be more aggressive, invading the dermis and subcutaneous tissue.
The most common symptoms include a rash or red, scaly, or crusty areas around the genital or anal area. These areas often itch or burn and sometimes bleed. Men often say the symptoms feel like “jock itch”. Occasionally, patients have no obvious symptoms or only have a slight increased sensitivity in the region. Pd generally affects postmenopausal women and men over the age of 50, with most cases occurring in individuals over 60.
Pd is typically slow growing and can take years to become noticeable and even longer to diagnose. Left unchecked pd tends to spread and can be deadly. This is why it’s critical to have any unusual symptoms in the genital or anal region checked by a physician.
Pd in women typically appears in the nipple, vulva or anus but can extend to the labia, mons pubis, vagina and thighs. Pd in men generally starts around the genital or anus areas but pd can appear in the scrotum, penis, groin, legs and perianal region. In the rarest of rare cases, pd has also been diagnosed on the axillae (armpits), buttock, thigh, eyelid, scalp and external auditory canal. Pd can occur in any of the apocrine-rich sweat glands.
When surgery is used it’s important to determine the edges and extent of the cancer. Sometimes skin grafts are also needed. If pd covers a large area, a reconstructive general, gynecological, urologist surgeon may also be a key player on your treatment team. Women with pd often use a gynecologic oncologist in their treatment.
Biopsies are the traditional way to determine the extent of pd. However, some doctors now use in vivo reflectance confocal microscopy (rcm) which can also help determine the extent of pd with digital “virtual biopsies”. Other treatments may include radiotherapy, co2 laser, topical creams or photodynamic therapy (pdt).
Due to the rareness of pd, and lack of clinical trials, chemotherapy has been used infrequently to treat pd. In the future this may change as doctors are now able to perfrom next-generation genome sequencing to look for mutations in tumor cells. Once a known cancer mutation is found, it can help doctors determine if any known drugs should be considered in the treatment. For more information: www.myempd.com