My Zuska's Disease: A Long Journey to a Devastating Diagnosis
UPDATE: This blog post was written on April 8th, 2016 and has been updated and revamped as of August 10th, 2020.
This is the most personal post I have ever written. When I wrote it back in 2016, I don't know what I was expecting to achieve - but I never anticipated the outpouring of messages I received from people experiencing the very same issues. The confusion, the recurring infections, the painful surgeries, the lack of answers or treatment options.
I remember feeling so alone and hopeless, much like I did at the beginning of my acne journey, only much worse. I spent most of my days crying and ruminating.
This was a very alienating experience, and due to its intimate nature I had an even harder time coping. Regardless, sharing my Zuska's journey, like sharing my acne journey, has helped me heal personally, and from the e-mails and messages I received, I can tell that it has helped others, too.
** WARNING: Graphic content, images & discussion **
Let's start at the beginning.
INFECTION # 1
It was January of 2014 or so. I remember there was a lot of snow on the ground and it was very cold. I was at work, and suddenly having these terrible breast pains in my left breast. I chocked it up to hormones, as breast soreness is not unusual for me. The pain was not like my usual breast pains, though. However, only having been off the pill for a few years, my body was still prone to all kinds of changes.
After a couple of days, the pain had still not gone away. It was constant. It physically hurt to wear a bra, and I felt completely side-tracked in my daily life. I couldn't focus on anything except the pain.
I came home from work one day and noticed that my breast was now swollen, inflamed, itchy, and red. The pain had now spread from my breast into my underarm area. Mortified, I also found a small, pea-size lump just under the areola. My first thought was breast cancer. Although panicking and concerned, I decided to wait it out and monitor for any further changes.
My period came and went, but the breast pain never went away.
A couple more days later, my breast was now extremely swollen, and I had a fever. Not knowing what else to do, I went to the Emergency room. This was around February.
I sat in the waiting room feeling stupid, like I shouldn't be there. The nurses treated me kindly, but I felt like they were thinking the same thing. People were there with broken limbs and concussions, and all I had was a sore boob.
I felt like an idiot, but I didn't know what else to do. I was scared and the pain was debilitating, and consuming my life.
In the semi-private exam room, I felt sick, weak, and light-headed. All I wanted was for the pain to go away.
The RN took one quick look at my breast and diagnosed me with a simple case of mastitis. I was given an antibiotic and sent on my merry way. She didn't ask me any questions about whether or not I was a smoker, or whether or not I was breastfeeding. And frankly, she didn't seem concerned. She was practically walking out the door as she was talking to me.
So, on her expertise, I didn't think any more of it. Just a simple case of mastitis. It will clear up with with some antibiotics.
And I thought, okay, phew, this is over.
I took the antibiotics, and as expected, my infection went away. I was thrilled, thinking I had seen the last of that immense and ineffable pain. I didn't care what it was, as long as it was gone.
INFECTION # 2
Unfortunately, the breast infection came back quickly.
I didn't understand. Why had it come back, and so quickly? So, I looked up more information on mastitis. I had heard of it, but didn't know what it was, really.
Mastitis is just a very general term for inflammation in the breast. It can be the result of many factors, but is usually caused during lactation, or as a result of smoker's gunk "plugging" up the breast ducts.
The pieces of the puzzle still did not seem to fit together. I'm not a smoker, nor have I ever been lactating or pregnant - why did I have mastitis?
This time, I went to see my family doctor, and he prescribed me another antibiotic - Norflaxacin. He didn't seem concerned.
I didn't get any answers or follow-up appointments at this point, only more medication to treat the infection. Again, the problem was out of sight, out of mind for the time being.
INFECTION # 3
Unsurprisingly, the infection came back a third time.
I should add that the lump never really went away during the antibiotics, just laid dormant for a month or two until the infection began again. It could always be felt just under the areola, but wasn't growing.
Somewhere around this time, although I cannot recall specifically when, my infected breast lump developed a fistula (sometimes called a sinus), which is an abnormal connection between two spaces. This is generally a pathway that develops so that an infection can drain - the body's way of getting out the bad stuff. This occurs in up to 50% of patients with abscesses. In my case, this fistula developed between the infected, pus-filled lump, and the outer layers of my skin.
I noticed this fistula developing when the lump produced a small, pimple-like whitehead of pus. For a while, I didn't know what it meant, and nothing changed. However one day, a shooting pain in my breast caused me to rush to the bathroom and check on it. It felt like someone had taken a needle and stabbed straight into my breast. It took the breath right out of me.
In the bathroom, I saw that the pus-filled lump had burst, and pus was draining out. It was more pus than anyone cares to see come out of a delicate area like the areola. I now had this little hole (about the size of a pin head), leaking pus and blood. I had to stuff my bra with tissue to prevent it from leaking through my clothes, and I had to change the tissue frequently.
I panicked and booked an appointment with my family doctor again. This time when we met, he agreed that it was cause for concern and he sent me to see a specialist.
Clearly there was something not being addressed.
INFECTION # 4
It took a couple of weeks to get in to see the specialist, and in this time the infection returned and I was on antibiotics again.
In the mean time, I also tried all the "natural" remedies I could think of (like iodine and colloidal silver). But nothing seemed to help.
My specialist was kind and knowledgeable, but I did not feel like he took my condition seriously. Little did he know we were in for quite a long ride together.
He sent me for an ultrasound, and the results came back inconclusive. My breast tissue was very dense (as is most breast tissue of a 20-something-year-old), so this complicated a proper visual. Breast ultrasounds and mammograms are unfortunately fairly useless in young women, according to him.
To put one of my bigger fears to rest, the specialist was able to rule out cancer as I didn't have any tell-tale symptoms like an orange peel-like appearance, scaliness, extreme swelling, etc., and had been dealing with this issue for over 6 months with no other serious progressions.
INFECTION # 5
Not knowing what else to do but get my serious infection under control before it turned worse, my specialist gave me another round of antibiotics.
The infection returned.
At our next appointment, we decided that the only way to see what was going on was to schedule surgery. This was at least the fifth time the infection had come back and I was on antibiotics. He knew, and I knew, that we couldn't keep up this charade with antibiotics and we had to try something else.
Unfortunately for both of us, it was going in blindfolded. We didn't know what to expect, since he couldn't see anything on the ultrasound. He didn't know what he was looking for, and so he didn't really have a plan.
I felt like I knew absolutely nothing going into the surgery. It was a very confusing and scary time.
The weekend of my birthday in November of 2014 I went in for surgery for a subareolar excision. The specialist essentially cut a small triangle of my areola to get to the infected duct. During this surgery, the visibly-affected duct and surrounding affected area were taken out.
I recovered well, and for several months it seemed like the problem was finally gone.
Under the scar tissue, I could no longer feel the lump. I was happy with my post-surgery aesthetics, and I thought all of this was in my past.
I was wrong.
INFECTION # 6
The pain in my breast returned in February of 2015. It was the same minor swelling, infection-like pain that resembled the pain of the first lump. Then the fistula developed again, and the same pimple-like whitehead appeared.
I don't even know how to express my level of devastation and frustration.
I was doing A LOT of searching online, as one is wont to do when they have no answers from doctors. But I never came across anything ground-breaking.
Whatever it was, the outlook did not look favorable, and a full mastectomy came up several times in my searches.
I loathed the idea of having my breast removed simply because of a non-cancerous infection that we couldn't find the cause of.
I pushed the idea out of my mind, sure that it couldn't possibly resort to a mastectomy.
LANCING # 1
On February 28th 2015, having been officially discharged as a "cured" patient after my surgery, I went to the Emergency room and had the new infected lump lanced and drained. I didn't know what else to do.
Unfortunately this did not help with the scar, which now widened and spread as the lancing left a gaping hole in my areola at least 3x the size of the hole before.
INFECTION # 7 ANTIBIOTIC # ??
And then the fistula came back again.
On March 4th I went back to my family doctor and was given the topical antibiotic Bactroban. It helped calm the infection, but it never got rid of the lump that was now always there.
At this time my doctor expressed the belief that there might be a small pocket of bacteria that simply wasn't being attacked by the antibiotic, which would explain why it kept coming back.
It was either that, or an antibiotic-resistant infection (which he was concerned I had caught during surgery). Neither were very promising outlooks.
INFECTION # 8
After the course of Bactroban, the fistula returned again. This time, unable to get in to see a doctor, and totally exhausted with the constant onslaught of antibiotic treatments, I was forced to drain the fistula myself. I had to do this in order to release the infection and to stop the fever and the pain. It probably wasn't the best thing to do, but I felt like I had no other option.
By now, my nipple was becoming disfigured by constant infection, healing, and re-opening of the fistula. I was also beginning to worry that the infections would lead to complete and irreversible nipple inversion.
By March 9th I was able to get back in to see the specialist and we talked about the potential for more surgeries. He expressed his concern that, due to the intricate nature of the breast tissue and all of the tiny, tiny ducts, and inability to pinpoint the cause, we may need to do a full mastectomy. This would be the only way to remove the affected ducts and tissue, to be sure we got it all.
Hugely upset, but not terribly surprised (I had been prepared for that possibility), I understood. He said he did not want to resort to that, just yet.
However, he also expressed the concern that continued minor surgeries and excisions would also likely leave my breast disfigured. It seemed like no matter which way he put it, I was going to lose much of my breast.
At this time the fistula was not visible enough, and there was no pus to drain (having drained and healed the day prior). This meant there wasn't much we could do at that point. So now it was a waiting game for the infection to return.
By now, I think I had partly come to terms with the complete removal of my left breast via mastectomy. I wanted the infections to stop. I wanted the pain to stop. No lump of fatty tissue was worth all this.
I continued using the antibiotic cream, 3x a day, and during this time the pain and the lump still remained. Around March 30th I reduced the Bactroban to 2x a day, remaining hopeful that this one last course would heal it all. Every time was the "last time".
INFECTION # 9
Unfortunately, June 5th I started feeling the pain again. On June 10th, the infection returned, and by June 11th the swelling, tenderness and itchiness had all returned, as well.
I was so uncomfortable all the time, always suffering from some kind of pain and being forced to go about my daily life as if nothing was wrong, as if it didn't feel like someone was stabbing me in the breast.
On June 17th I went back to my family doctor, and this time he gave me Cefzil and told me to try using a tea tree oil product on it. I was still using the Bactroban 2x a day, Cefzil 2x a day, and tea tree oil spray topically.
On June 27th I finished my topical and oral antibiotics. For the next month, still using the tea tree oil spray, I had no problems. I felt as though the tea tree oil spray had been just what I needed. It was bacterial, after all.
Again ... so I thought. I only got two short months of relief, this time.
INFECTION # 10
Unfortunately, around August 27th of 2015, the pain, swelling and infection all came roaring back.
I know what you're thinking, I'm getting tired of this, too.
You can imagine how upset I was, having to deal with constant infections, no answers, no real treatment options, and never quite feeling like myself. I always had this stress in the back of my mind. I always had this pain.
September 1st, the fistula formed once again. On September 2nd I went back to my family doctor and was put back on Cefzil (like, this is getting ridiculous ...)
At this time, I was 100% convinced that this bacteria was antibiotic-resistant, and so I made another appointment with the specialist to get a bacterial culture/biopsy done.
On October 5th, 2015 the specialist attempted to get a culture. Unfortunately the fistula was again not active enough, and so after four excruciating prods in the nipple with a massive needle, we had gotten nowhere.
He requested that I wait until the infection came back, and come in to see him ASAP to attempt another culture.
INFECTION # 11
Around October 30th the pain and swelling came back, but no fistula.
On November 3rd, the fistula developed, and so I quickly made a call to my specialist. Unfortunately he was in surgery all week. I was now stuck with this horribly inflamed and painful breast infection and no idea what to do for relief.
I tried to get into my doctor's office for another prescription (feeling the fever coming on), to no avail. Apparently my lucky fistula decided to come back during a professional development weekend when all of the walk-in clinics were closed and the only place I could go was the Emergency room.
I didn't want to go back there. So I decided to go to the store and get some Polysporin with antibiotics to hopefully hold off the infection until I could see the specialist. This seemed to help somewhat, and kept the situation under control for the time being. The fistula drained and healed as usual.
While I waited for my appointment, I once again took to the Internet in an attempt to understand my condition. This time I came across a forum that discussed recurring breast infections resistant to antibiotics. On this forum over a dozen women had come together to discuss their breast infection that kept coming back despite countless surgeries and courses of antibiotics. The whole outlook seemed so terribly futile.
Women from all over the world had a similar prognosis as I did, and each and every one of them had stumped their doctors. Many of these women claimed to have been dealing with these same breast troubles for 15+ years! I cringed at the thought of having a breast infection requiring antibiotics every 2 months for the rest of my life. However, most of these women only experienced a flare-up once a year, so mine was an extremely severe case. At this point I was so beyond hopeless and confused.
I was baffled. My doctors were baffled. Nobody had any clue what to do next. I don't think I have ever felt so without hope as I did then.
During one point of incessant late-night scrolling for answers in the dark corners of the web, I went to Google Images, and it was here that I accidentally stumbled across the answer to everything.
Most photos I had seen before didn't quite look like my situation. They all differed somewhat slightly, mostly because most breast cases like mine are cancerous and cancer in the breast has a very distinct appearance. However, when I came across this image, something clicked. I held my breath as I clicked on the picture.
This image looked strangely similar to what I was dealing with, more similar than any other image I had ever come across. Although the abscess and fistula were higher up, to the side of the nipple, the picture looked identical to what I had. I cannot even begin to tell you how hopeful I was just to have found this photo on pure chance, and even better, it was linked to a website that had a name that I could research:
Although, research on Zuska's disease was not plentiful at the time. I needed to do a lot of investigative work. If you search Zuska's now, you get dozens of results on Google - many reputable and scholarly journals and articles, diagrams and treatment information. This was not the case back then. I found one article on a scarce website.
However, what I did find was extremely helpful. My questions began to get answered, my confusion was alleviated, and I was able to come to terms with a treatment plan a little easier.
Zuska's disease is a rare but painful breast disease. Because very little is known about the disease, it is often misdiagnosed and inappropriately treated (multiple surgeries, antibiotics, draining - sound familiar?). It is not related to breast cancer, but it is still a serious condition. Also known as lactiferous fistula or Zuska-Atkins disease, it is a recurrent disorder characterized by draining abscesses around the nipple. If infections are left untreated it can lead to nipple retraction, or worse.
It is a form of epithelial squamous breast metaplasia which is essentially an overproduction of epithelial squamous cells, which then results in the blocking and plugging of the breast ducts. Keratinizing squamous epithelium replaces the lining of one or more lactiferous ducts, and right into the subareolar tissue. When the breast ducts become obstructed by the keratinaceous debris, it may cause the ducts to tear. The contents of the duct then spills out into the surrounding breast stromal tissue, resulting in a chronic and active granulomatous inflammatory reactive state.
This explained the lump. This explained the constant and recurring infection.
Symptoms of Zuska's disease are a bacterial infection, swelling, subareolar mass, a painful nodule under the areola, a draining fistula, and possibly discharge from the nipple (the only symptom I hadn't had).
Now, what about treatment?
Even though this is a benign disease, it is still very serious and very painful. And unfortunately, treatment requires dramatic measures. The infection, a secondary symptom, must be treated with antibiotics, and sometimes incisional drainage procedures can relieve the symptoms, but fails to address the fundamental and recurrent problem. Not to mention, also lead to scarring, and nipple and breast distortion, all without curing the problem.
The bottom line is that if this keratinizing epithelium is not removed, it is highly likely that the problem will continue to reoccur. Zuska's disease must be treated with surgery, specifically surgical excision of the fistula, all of the retroareolar fibroglandular tissue, and any ductal tissue within the nipple. This procedure is sometimes referred to as radial elliptical incision with primary closure. Studies at the Cleveland Clinic show that this procedure has around a 91% cure success rate and 95% satisfaction rate with the appearance of the breast post-surgery.
Everything fit. Everything. It all made sense. Unfortunately, although I finally had a name for my problem, and an understanding of it, the treatment options were no better than what they were prior to knowing these things.
Regardless, this was a rare and radical problem, and I needed to take drastic measures to get rid of it. I was willing. The way I saw it, it was either: run the risk of developing antibiotic resistance and potentially dying from an otherwise easily treatable problem in the future, or have part of my breast removed. Seems like an easy choice when you put it that way, right?
I at least had a game plan. I could rest easy knowing I had a next step in the process, and that gave me some hope to cling to.
On November 23rd, 2015, I met with the specialist once again. It was a casual meeting, with a medical student also present. However the appointment once again left me walking out in tears. I had so much hope for a game plan going into the meeting, and left feeling more hopeless than when I began it all.
My doctor acknowledged that Zuska's was my probable diagnosis, but that it did not change my situation. He also addressed the studies from the Cleveland Clinic and discussed how the Clinic is a top hospital where the very wealthy from all over the world go for the best treatment, and that there is a certain level of expertise to be expected there. Thus, the results regarding a 91% success rate and a 95% aesthetic satisfaction rate may be slightly biased.
Again, my doctor expressed concerns with another surgery, regarding taking out too much breast tissue. He didn't want to jump right into a full mastectomy, and of course, I don't blame him. Taking the breast of a 25 year old without cancer seems extreme, and it wouldn't be his first, or his second option.
The radial elliptical incision with primary closure surgery that was mentioned in the literature wasn't an option. He expressed that this would result in him taking out a lot of tissue, which would no doubt leave me with a "caved in" or sunken breast, which would make me very unhappy.
He wanted to try something else, first. He talked about doing some kind of surgery that used a long instrument to follow the duct as far down as he could to remove a much larger chunk of it, and said that this would unfortunately leave me with a massive scar extending far outside of the areola and into the regular skin tissue.
I said I didn't care, whatever you need to do. At the same time, I only feared it wouldn't work, it wouldn't be a solution, and I would just be back in there in another few months booking another surgery.
I wanted the surgery that the doctors in Cleveland had performed, but he assured me that I wouldn't be as happy with the results as I thought I would be. What could I do? He is the specialist, and he knew better. I had no choice but to let him make the call, because I wanted to know as much as he did that we had exhausted every avenue prior to radical measures.
First, he suggested an MRI. He hoped that he might be able to pin-point some inflammation, although he also acknowledged it may also prove fruitless as MRIs can be extremely sensitive to any inflammation at all, even that leftover from my last surgery.
However, the MRI machine for breast imaging was broken, and had been for several weeks. So that didn't help us much with that avenue. Just my luck, right?
Feeling like we had no other option, he suggested that I wait until the infection came back again, and make an emergency appointment for a last-minute surgery.
So I left his office feeling completely overwhelmed with emotion, confused and unsure. Another surgery. I knew it was coming so I don't know why I was so unprepared to hear that.
That very same day, I felt the infection beginning again.
INFECTION # 12
I had a huge breakdown. I called my husband and cried to him on the phone in the parking lot. He echoed my pain and frustration, although I don't think he understood the situation. He couldn't grasp the rarity of my diagnosis, nor the fact that something that seemed so menial could result in such a serious surgery. Despite his and my anger and our frustration at wanting this madness to end, I knew he would support whatever decision the specialist and I made.
I cried most of that day. I went to work crying, my eyes bloodshot, and I had to write my employers a very uncomfortable letter spelling out the details of my condition, which meant I would have to be off work very last-minute. It was a really, really, really hard day for me. I think this was the day the weight of the situation truly hit me.
COMING TO TERMS
The answer was clear - I had to endure these surgeries no matter what, even if it did result in a full mastectomy and its potential consequences. These were realities that were not only possible, but likely.
I started thinking about all the people in the world who have had their breasts removed. I thought of their brave, smiling faces, despite the hardships and tears they've no doubt been through. And they were okay. Their life went on, and I was going to be okay, too.
No matter what happened, a mastectomy wouldn't stop me from living my life happily and to the fullest. It wouldn't stop me from writing, playing with my dogs, having movie nights with my husband, going on road trips, sipping green tea and reading a good book - all of the things I enjoy in life, I was still able to look forward to no matter what happened.
Instead, I tried to use my situation as an opportunity to model how to be strong in the face of bad news, how to be positive and hopeful and persevere, how to see that what seems devastating probably isn't all that bad. I would come out of it a better person. I always do. Everything bad that has ever happened to me has made me stronger, wiser.
I decided to face this problem with a fierce promise to be hopeful, to throw myself in 100% and to not look back, to not be afraid or worried, but to know with absolute certainly (as much as we can know anything with absolute certainty) that even in my worst case scenario, it was all going to be okay. I was going to take this on and I'd be damned if I was going to let something like this take any moments of joy away from me.
And I think I kept my promise to myself. I didn't think about it when I didn't have to, and I didn't worry about it when I did have to think about it.
Around November 27th, about a year after my first surgery, the infection formed a pimple-like lesion, and again the fistula opened and drained. I couldn't call the specialist for surgery because it wasn't the infection he was waiting for. It was too mild. Imagine that. It had to be really inflamed, and really infected. So I had to wait for the next one.
In the mean time, I refused to make another appointment for another antibiotic, so I decided to try another natural remedy.
I have long known about garlic's potential as an alternative to antibiotics, but I was skeptical it could help such a severe case as mine.
Many of the chemicals in garlic have been studied for killing bacteria. One study, published in a 2011 issue of Applied and Experimental Microbiology, used a variety of biochemical techniques to examine how some of the compounds in garlic interacted with bacteria and found that garlic was effective against the bacteria C. jejeuni. Another study, published in the Journal of Medicinal Food found that garlic (allicin) was able to kill some pathogenic oral bacteria, such as S. mutans and P. gingivalis.
If you are looking to consume garlic for antibiotic purposes, the University of Maryland Medical Center recommends 2 to 4 cloves of garlic or 600 to 1,200 mg of aged garlic extract daily.
Feeling like I had nothing to lose, and knowing I had everything to gain, I ran to my local health food store and stocked up on garlic supplements. I figured I could get more garlic into my system this way than chewing on raw garlic all day, which was far from enjoyable.
I remember crying in the parking lot and thinking, this isn't going to work - just more money down the drain, more effort and hope lost. But I took them anyway.
At this point I just wanted it over, at any cost. I was tired of living with pain and infections, living and coping with the stress and the fear of the unknown and the repercussions. I wanted my life back.
THE GARLIC WORKED?
Around Christmas 2015 the problem still had not manifested again, so I was able to enjoy the holidays. I was waiting, on eggshells, expecting the infection to come back right smack dab in the middle of Christmas holidays when the possibility of surgery was nil.
But then January 2016 came and went. February came and went, too. Then March. Still nothing.
I couldn't believe it. Here we were, preparing for another invasive and disfiguring surgery before we resorted to a mastectomy, and it just ... cleared up! Went away. Poof. Gone!
I still had twinges of pain in my breast right under the scar, but it usually coincided with my menstrual cycle and didn't last long or develop into anything. There was some occasional discomfort and perhaps minor swelling, probably due to pressure on the scar tissue, but the lump hadn't returned and there had been no fever, no draining, and nothing truly alarming.
I couldn't believe it. I counted my lucky stars, kept up with my garlic supplements religiously, and prayed the infection would never come back.
As of 2017 I am no longer taking the daily garlic supplement.
At the end of the day, I am still confused as to what I think helped most? It's really hard to say. I had so much going on, with so many different antibiotics being used, so many different remedies tried. I'm sure they all contributed, at least somewhat.
However, I feel confident that it was the consistent use of garlic supplements every single day for weeks and months that helped to get the stubborn infection under control.
UPDATE: As of 2020, I have not had any further issues or signs of re-occurrence. I consider myself so incredibly fortunate, and hope that anyone dealing with the same pain experiences relief soon.
If you're dealing something like this and feel you have nobody to reach out to, please remember that you have me. I will happily lend a listening ear.
<3 So much love to all of you who have reached out to me over the years, sharing your Zuska's stories, as well. Keep fighting.