Paget's disease
Breast Cancer
Breast Cancer
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inflammatory | paget's disease | pregnancy

Paget's Disease

By Lynne Gidish

Paget’s disease is one of the most missed and misdiagnosed of all cancers. Once extremely rare and acknowledged as the sole domain of menopausal women, it’s now affecting more and more - and younger and younger - women. If you’re being treated for an eczema or sore on the nipple that just won’t go away, consult a breast specialist immediately!

Paget’s Disease of the breast is an eczema-like change in the skin of the nipple, and 9 out of 10 women who have this have an underlying breast cancer. The breast cancer may be invasive i.e. it has spread beyond the breast ducts, but in many cases it is localised in the ducts. In other words the cancer spreads along the ducts and spills onto the nipple instead of sideways out the ducts into the breast tissue. Paget’s disease occurs in about 1–2 out of every 100 women with breast cancer. It usually occurs in women in their 50s, but is now affecting women who are much younger, according to Dr Carol Ann Benn, a surgeon with a special interest in breast health. “The first symptom is usually an eczema-like rash, often with crusting, oozing, weeping or bleeding,” she explains, “The skin of the nipple and areola may be red and inflamed and some women have an itching or burning sensation. Paget’s disease is often confused with other skin conditions such as eczema, dermatitis or psoriasis, which can makes it difficult to diagnose. That is why anyone presenting with these symptoms needs to undergo a thorough clinical examination, mammogram and ultrasound (sonar) as well as a skin biopsy regardless of the clinical findings.”  Pagets disease fascinates me because there must be something in the genetic make –up of the person that causes the cancer to travel up the ducts and spill onto the nipple .Often these women have extensive areas of high grade pre-invasive breast cancer (DCIS) and only small areas of invading breast cancer. This preinvading breast cancer is often Her 2 neu positive. We do not understand the significance of this. The treatment depends on the size of the invading breast cancer and the personality of the invading breast cancer. So the story with Pagets is the earlier it is detected the less chance of their being invasive breast cancer and the better the prognosis. Pre-invading breast cancer has a 99% cure rate.

Paget’s disease is one of the few types of breast cancer that should be treated with a mastectomy followed by immediate reconstruction. “While breast conserving surgery ( i.e. a lumpectomy) has now become the treatment of choice when possible for most breast cancers,” says Dr Benn, “In the case of Paget’s  it is not an option. This is because the cancer can often arise from any area of the breast. Any form of further treatment like chemotherapy and radiation is dependent on the findings during surgery.”

This is an important lesion to recognise as more than 90% of women with Paget's disease have an underlying breast cancer. Paget's disease is a lesion that looks like a scaling oozing, weeping, crusting manifestation of the skin of the nipple an areaola complex. It is sometimes associated with a bloody nipple discharge (discharge may also be purulent). It affects one breast more commonly than both breasts and mimics eczema. When it presents, 20% of patients will have no other breast problems clinically and up to 50% will have an associated breast mass. It is very important to do a thorough clinical examination and a breast utrasound (sonar) and or mammogram depending on the age of the patient. Occasionally, melanoma of the nipple can resemble this condition.

Any person presenting with this condition must undergo a skin biopsy irrespective of the finings on clinical examination, underlying mammogram, or breast sonar. Any non-healing lesion of the nipple and areaola that is being treated as exzema should send off warning bells and a skin biopsy should be perfomed or the patient referred to a breast specialist. Page's disease can also be caused by breast cancer, or as part of an inflammatory cancer where the whole breast looks hot, red and inflamed, or as cancer cells that have not spread (ductal carcinoma ins situ.) Occasionally a small area of ulceration can occur.

It is one of the few types of cancer that should be treated with a mastectomy and immediate reconstruction as the cancer can oftern be arising from any area of the breast. Indications for chemotherapy and radiation therapy must be taken on the findings at surgery.

Auerika Haasbroek (37) is a married mother of an eight year old son.
In October last year I went to my GP as I was suffering from sinusitis and asked her to check my right nipple which was quite itchy, crusty and a lot redder than the other one. She said it could be eczema, prescribed a cortisone cream and suggested I come back if it hadn’t cleared within ten days. The itchiness went away and the redness faded and when it  returned I  reapplied the cream and it went away again. In December we went on holiday and my nipple started bleeding slightly. As I was being treated for eczema, I wasn’t concerned and just applied more cream. In February I went back to my GP for my annual pap test and showed her the nipple again. By this stage I was wearing a tiny plaster to stop the scab from being constantly scuffed off. She referred me to a breast clinic to have it checked while undergoing my routine mammogram and ultrasound. Expecting to hear that everything was OK, I was completely gobsmacked when the doctor took one look and said, ‘Yes, this is cancer’. I could hardly see any difference between the left and right nipples! That’s when I first heard about Paget’s. I couldn’t believe it. It was the last thing I ever expected- there’s no cancer at all in my family. When my mammie and ultrasound came back clear, it gave me a glimmer of hope that he was wrong but I was told that this is normal for Paget’s. A core needle biopsy then confirmed the diagnosis, as did the specialist I consulted  for a second opinion. I was in total shock. After a lot of careful thought reviewing my different options I underwent an elective bilateral mastectomy with reconstruction in May. They managed to get it all away, and because the associated tumor was very small no further treatment was needed. The process of reconstruction will only be completed in March next year which means the whole experience is still very much part of my life. I’m dealing with it to the best of my ability. Cancer has a way of making you take an inventory of your life and right now I’m looking forward to making the most of the second chance I’m so thankful to havebeen blessed with.

Lize de Jonge (30), single
Nine years ago I noticed a dry patch on my left nipple and went to my doctor who diagnosed eczema and gave me cream to apply. For the next three years it was always present - sometimes better; at other times worse- and I was frustrated by the fact it never went away despite religiously using the cream. When I was 24 I read about my symptoms in an email I received and broke out in a cold sweat. It didn’t mention eczema but warned women to be on the lookout for a type of breast cancer called Paget’s. I got extremely nervous and  immediately referred myself to a dermatologist. He did a biopsy and called me a few days later with the results. I was devastated by his approach. To say to a young woman in her twenties that ‘you’ve got cancer and you’re going to lose your breast so come in next week’ just wasn’t on. I kicked into survival mode- I chose fight not flight – and went to a breast specialist for a second opinion. Finally everything started falling into place. I was sent for scans and  tests and knew that at last I was in the right hands. I had a mastectomy soon afterwards with immediately reconstruction, followed by a full course of chemo. Since then I’ve gone for checkups every six months which have always thankfully come back clear.  Post-op I had to learn to fall in love with my body all over again, which took time. But I was young and resilient which definitely pulled me through even during the bad times, as did support from family and friends. Going through something like this makes you more of who you’re meant to be, and life becomes a little more precious too. Today I prioritise my health because I feel good when I respect my body. I also never take being healthy for granted and never assume that the medical profession knows it all. I’ve learnt to listen to my body and when something’s wrong go for a second opinion if I’m not satisfied with the first. It’s my life that’s at stake after all.  

Petronel Greyling (35) married mother of two boys aged 5 and 4
In December last year I noticed a small sore on my left nipple. I was given antibiotics by my doctor and it went away. It came back in February and I was put onto antibiotics once again and was also sent for a mammie and ultrasound. When a calcification was picked up in the breast, I then had to have a core needle biospy and when the results came back I was told I had Paget’s. I had no idea what it was, no idea that it was cancer and was completely shocked and terrified when it was all explained to me. There’s no history of breast cancer in my family and this form is extremely rare which made it even more difficult to believe. What further confused me is that like most people I’ve always associated breast cancer with lumps in the breast and all I had was a sore on the nipple. I had to undergo further tests to see if it had spread, which it thankfully had not. In April I had a mastectomy followed by reconstruction. Because it was picked up so early and was localised in the ducts I luckily didn’t have to undergo chemotherapy or radiation. It was an extremely difficult time but as a wife and mother you have to accept things that happen and deal with them - there is so much else to live for. I never really worried about dying because I knew right from the start that if they got it all the prognosis is excellent. I’ve successfully managed to pick up the pieces of my life, so much so that when I look back, at times it almost seems like it was all a dream, which never really happened. But of course, every time I get undressed, that’s my daily wake up call. Despite everything I’ve been through, nothing’s really changed- I’m still the same person I’ve always been, just filled with gratitude for the clean bill of health I’ve been given,  and living and enjoying every minute of my life.