Pemphigoid Gestationis: A Patient’s Story
Pemphigoid Gestationis is a rare autoimmune blistering skin disorder that affects an estimated 1 in 40,000-50,000 pregnancies in the United States each year. United Kingdom figures put it at 1 in 1-2 million people. You could say that each of us is one in a million! No one wants this; most have said that they wouldn’t wish it on their worst enemy. This is my PG story.
May 10, 2013 was the first time I had heard of pemphigoid gestationis (or gestational pemphigoid, as my doctors all called it). I was nine days postpartum with my first miracle son. The pregnancy had been a struggle with my doctors who had advised me to abort him at 17 weeks and to not get pregnant again, as I would “never be able to carry a baby to viability.” When my son was born at 38 weeks gestation by emergency C-section because his heart kept stopping with contractions 16 hours into labor, he came out crying. My husband and I, understandably, breathed a huge sigh of relief. My next thought was, “It’s over; I can just be a normal mom.” Sadly, that wasn’t to be, either.
I tried to ignore the itch as much as possible, but it just seemed to be getting stronger and more widespread.
Within 36 hours of the birth, my palms were itching, and there were little, red bumps all over them. I asked every nurse or doctor that came in my room about it. One nurse told me that it was probably because I was allergic to lanolin, as I am allergic to wool. I stopped using the lanolin. It didn’t help. Everyone else just ignored me or said, “You are allergic to something here at the hospital, it’ll go away when you go home.” Once home, I almost immediately took a shower, hoping to wash away whatever it was that was bothering me. That didn’t happen.
On Sunday, we were trying to get used to having a newborn at home. I tried to ignore the itch as much as possible, but it just seemed to be getting stronger and more widespread.
On Monday, blisters started to form on my hands and thighs and in my belly button. I stopped taking pain medication, the only new thing that remained from the surgery and hospital. While my pain increased slightly and my ability to move became slightly less, the rash showed no signs of easing.
On Tuesday, I called my obstetrician. Her nurse told me to go to the emergency room. I was unwilling to drive the hour and twenty minutes to the larger city hospital where I had delivered my son. So, leaving him at home with my mother who had come to stay with us to help out, I went to our small, rural ER, where I was told, “It looks like an allergic reaction.” They gave me a steroid shot and sent me on my way.
As he was walking out of the room, he told me that I should probably stop breastfeeding my son because of the high dosage of prednisone he was prescribing.
On Wednesday, I tried to tough it out, hoping that the steroid shot would help my immune system get the rash under control. Thursday morning, my newborn had his first appointment with Dr. Julie Rosa, a family medicine doctor at the local clinic attached to the hospital. She checked him over, declared him healthy, and then turned and asked what was going on with me. I told her what everyone had told me. “I guess it’s an allergic reaction, but I don’t know what to do.” After asking a few more questions, such as when it had started, where it had started, if it itched or hurt, etc. she drew around a few of the blisters, gave me a prescription for an antibiotic to ward off infection from all of the open wounds, and instructed me to come back during open clinic hours the next morning.
I did as I was told, and after signing myself in, the receptionist, using a tissue, picked up the pen I had used and threw it in the garbage. In the waiting room, I was stared at and asked, repeatedly, “What’s wrong with you?” Once in a room, three doctors from the clinic stood around looking at their smartphones, a textbook, and me. One of the doctors wrote down their consensus diagnosis: gestational pemphigoid. Doctor Julie then jumped into action, calling dermatologists in the general area, trying to find me an appointment for that day so I didn’t have to suffer through the weekend. She found a dermatologist’s office that was nearly 2 hours away and would close in about 2 hours and 15 minutes. The doctor didn’t have an appointment open, but his physician’s assistant agreed to see me. We hurried out, stopped at the house for snacks, frozen breast milk and some more diapers, and headed down to the new doctor’s office. We got there with a few minutes to spare, and I was an emotional mess. This receptionist kindly took my hand in hers and said, “We will get this figured out, and we will get you better.” I needed that reassurance.
The dermatologist stepped into the room for a moment after the PA had taken down all the pertinent information from me, took one look at me, and said, “Yes, definitely Gestational Pemphigoid.” He instructed the PA to do the biopsies to confirm and told her which medications and dosages to prescribe. As he was walking out of the room, he told me that I should probably stop breastfeeding my son because of the high dosage of prednisone he was prescribing.
I left the office that day hopeful and crushed. Breastfeeding, while not going flawlessly, was working; it felt like this was the only part of childbearing that my body was actually allowing. As I struggled in the back seat of my car with a nipple shield, a nursing cover, a crying, wiggly newborn, and my own emotions, tears slipped down my face uncontrollably. Indeed, over my four months on prednisone while postpartum, the vast majority of times that I lost it were over breastfeeding or pumping. This is common for new moms, but prednisone definitely doesn’t help those postpartum (or pregnancy-related) hormone fluctuations! I decided to seek other opinions before stopping, and I am glad I did. I was working as a pharmacy technician at the time and had access to multiple pharmacists. In addition, I sought Dr. Julie’s advice, who now oversaw both my son’s and my own primary care. All of them concluded that the benefits outweighed the risks, even those for whom it would have been easier to say, “no, stop,” since that would mean I wouldn’t have to pump when I returned to work. I ended up pumping and dumping while taking a split dose of 80mg a day (40/40), but resumed once I was able to take prednisone just once a day. In PG literature, it suggests that breastfeeding is helpful for resolution of the active disease. In my interactions with over 350 PG patients in the Facebook group for PG that I help administer, I would say most of us breastfed for at least a couple of months.
It took four months to taper off prednisone completely, and I have not had to go back on it. That is not to say I have not had any symptoms of PG. Like many PG ladies, I had monthly, localized “mini-flares” that coincided with my menstrual cycle; most of the time these do not require oral medication. Ironically, they stopped when I got pregnant again with our second child, also a boy, who despite the reported 90% recurrence rate in the literature, was indeed a skip pregnancy. In our group, which has many more respondents to this question than the largest PG published study (87), our numbers run at about a 65% recurrence rate with another 15% not needing oral medication to manage the flare.
“We will get this figured out, and we will get you better.” I needed that reassurance.
Because I didn’t have active PG while pregnant, I didn’t have to worry about the effects of PG on my sons in utero. However, that is not the case for many women. A PG pregnancy is a high-risk pregnancy. According to studies, the most common effects of PG on the unborn child are preterm birth (usually at least 35 weeks), intrauterine growth restriction, and placental failure late in the pregnancy. The duration of the disease affects this the most; i.e. if its onset was in the first or second trimester, as opposed to the third. Although the miscarriage/stillbirth rate is the same in PG pregnancies as in the general population, it is important to have close monitoring of the baby through ultrasound and non-stress tests, especially late in the pregnancy, to ensure that the baby is doing well. Prednisone use during the pregnancy can also lead to gestational diabetes and preeclampsia and/or eclampsia, which pose other challenges. Also, about 3% of PG babies are born with a mild rash, which usually resolves on its own in a few days and does not seem to bother the babies at all.
Despite doctors’ and researchers’ claims that there is no need to study this disease because it only affects pregnancy, which is preventable, there are long-term effects on PG patients. As previously mentioned, many women get monthly reminders with their cycles. Birth control options are limited because the use of combination hormonal birth control can frequently cause flares. As with all autoimmune diseases, the likelihood of developing others increases. For PG patients, the most common are Graves’ disease and Hashimoto’s disease, both of which are thyroid-related.
If you are or have suffered from pemphigoid gestationis, help and support is available on Facebook at https://www.facebook.com/groups/PemGest. If you are a caregiver for someone with PG https://www.facebook.com/Pemphigoidgestationis/ is a community page with links to studies, as well as a place to ask questions. Of course, the IPPF is also willing and able to answer questions as well.