Saturday, October 1, 2016

Henoch-Schonlein Purpura: It's Just As Cute As It Sounds

It started three weeks ago when Zuzu ran away from soccer practice and ended up with a bunch of chigger bites on her legs and bottom.

She had a bath right away when she got home that night, but the next night she had a baby sitter and David and I went to parents' night at the girls' school. So it was Friday before I really looked at her legs/bottom again, and I was shocked at how red and angry the bites looked. I treated them with Benadryl gel and some Neosporin over the weekend.

On Monday she had a doctor's appointment to get her four-year-old vaccinations, plus a flu shot. I pointed out the bug bites on her legs to the doctor and he didn't seem concerned. He said I could keep using Benadryl or Neosporin.

On Wednesday we got a call from her school around lunch time that she had a fever. I picked her up and she slept all day. I figured it could be related to the vaccinations or it could just be a back-to-school virus. David stayed home with her on Thursday. She didn't run a fever again, even without tylenol, and she didn't complain about her bites. She wasn't scratching or messing with them at all, which surprised me, because they didn't seem to be getting better.

On Friday, our friends Julie and Cate flew in from Minnesota for a weekend visit. The girls played together on Friday, and I showed Julie how bad Zuzu's bug bites looked. Some were getting better, but others almost looked like they had bruises around them. But I reminded myself that the doctor had just seen her on Monday and hadn't been concerned. Plus, I was taking extra care to keep them clean and medicate them, and I was sending her to school in leggings even though it was warm enough for bare legs, just because I wanted to protect them from being exposed to more dirt.

On Saturday, all the girls played hard at the Magic House in the morning. That afternoon, I put on Mr. Roger's Neighborhood so Julie and I could have some uninterrupted conversation. Zuzu fell asleep, which was unusual. She wasn't really herself for the rest of the day, either. She occasionally complained that her tummy hurt, and didn't have much of an appetite. We went to Forest Park that afternoon and all the girls ran around and played, but Zuzu complained that her legs hurt. I figured she was just tired because they'd been up  late on Friday.

On Sunday, we said good-bye to Julie and Cate and Zuzu seemed okay, just kind of droopy. But she complained about her ankle, and I realized that it looked bruised and swollen. I made David look at it, and we wondered out loud if kids could sprain their ankles? It seems like their ligaments are so stretchy that it's pretty rare for a preschooler to have a sprain, right? I put ice on it and propped her foot up on a pillow while reading to her in bed that night.

She seemed to be feeling better on Monday, but I was getting worried that the bug bites didn't seem to be healing properly. Some of them seemed to be getting worse, and instead of looking like bug bites, they were definitely morphing into some kind of rash.

By Wednesday, we were back at the doctor. He took one look at her, said that it was an allergic reaction plus a mild staph infection. He prescribed an antibiotic ointment to be put on three times a day.

We ran through the little tube in about two days because the bites/rash were all over her legs, the backs of her arms/elbows, and her bottom. On Friday, I called the pharmacy to get a refill, but my insurance didn't want to pay for a refill so soon, so I had to call the doctor's office, and then the pharmacist ended up having to call my insurance, and it was Saturday afternoon before we could pick up another tube of it. Over the weekend, I continued to smear it on her every morning and every night, plus once after school, and the rash was not improving. In fact, there were red lines where the elastic of her undies seemed to have irritated her skin (something that had never happened before). I was having her sleep in loose-fitting pajama pants and no undies, but it still hadn't improved.

On Sunday she got stung by a bee while outside eating a popsicle. She'd been stung last year, too, so I fussed over her while she cried and got her an ice pack and put some lavender oil on it and let her watch PJ Masks. I wasn't too concerned about her having an allergic reaction or anything because we'd already been through this once. But this time she got an angry red rash in almost a square shape a few inches across, all around the bee sting. Plus she took off her shoes that evening and told me her feet hurt and she had red spots on one toe and the arch of the other foot. I started wondering if she could have gotten something else, or if I had assumed they were chigger bites when maybe they were some other kind of terrible insect (watching Stranger Things around this time was probably not great for my imagination). I also started googling symptoms of all the things that really scare me: lymphoma, leukemia, MRSA, meningitis...

The only thing that kept me from losing my mind with fear/worry is that she never ran another fever, and she never complained about the rash--never said it hurt or itched or tickled. She was completely unfazed even though I wanted to cry when I looked at her legs and bottom. It was the weirdest thing.

Monday morning, I called and spoke with a nurse. She thought maybe it could be HFM if I was seeing red spots on her feet, even though I explained that they didn't look like HFM when my other daughter had it. These spots weren't like blisters or scabs--they were more like bruises. She told me to give her Zyrtec for the bee sting and keep using the Mupirocin ointment and to call back Wednesday if things didn't improve.

Monday night when she took of her pants before the bath, I gasped. In addition to the bee sting area still being red and angry, she had red lines around her legs where the elastic of her socks or bottom of her leggings had been. This was NOT normal. We were coming up on three weeks from her last soccer practice, and while some bites seemed to have healed and faded, others were looking worse, and I just knew something else was going on.

Tuesday we were back at the doctor. This time David came with us because I was kind of freaking out. The doctor assured me that she wasn't contagious, it wasn't a flesh-eating bacteria, MRSA, or leprosy. But he said it was "very unusual." He referred us to a pediatric dermatologist and he didn't let us leave his office until his receptionist had called and set up an appointment for us on Thursday afternoon.

After leaving the doctor, I sent a photo of Zuzu's legs to my friend Erin, telling her I was freaking out and that we'd been referred to a specialist at Children's.

"That looks like a rash my sister had when she was little," Erin replied. "Allergic purpura."

I started googling.

It turns out that Allergic Purpura is now called Henoch-Schonlein Purpura. It's named after the two German doctors who discovered it, but "purpura" means small blood vessels are leaking into the skin. I read WebMD, the Mayo Clinic, and the Johns Hopkins Vasculitis Center website and just KNEW that's exactly what we were dealing with. All the pieces fit. She had every single symptom, though I realized that I had probably neglected to report her tummy ache or complaints about her legs hurting to our doctor. When he asked if she was complaining, I'd been so focused on the rash that I had kind of blown off her mild appetite loss and occasional whining about her legs hurting--poor little punkin!

Thursday afternoon, I took Zuzu to Children's Hospital and the dermatologist, three residents, a med student, and a nurse all gathered in the room to see her "classic presentation" of Henoch-Schonlein Purpura. The dermatologist also told me (unprompted) that our pediatrician "knows what HSP is" and "is an excellent physician" but that the bands around her ankles had thrown him off (which is also what freaked me out) so he wanted a second opinion.

It was a little funny when the first resident came in because I'd prepped Zuzu by telling her that the doctor was a woman; however, the resident who talked with us first was a tall Indian guy with a five-o'clock shadow. Zuzu did a quick double-take and then said to me loudly, "Mommy, is that a boy?" so I had to explain her confusion.

She was an awesome patient, letting them examine her and sitting quietly during my conversation with the doctors. I was really proud of her and how well behaved she was all afternoon.

So here's the thing: HSP is the inflammation of blood vessels that leak into the skin, causing what looks like a rash with yucky raised bumps, but they are actually under the skin rather than on the surface. The rash doesn't itch or hurt, but it can cause pain in joints and the gut as vessels leak there and cause swelling and lack of appetite. In some cases, it can cause kidney damage. There is no known cause for HSP. It's sometimes associated with upper-respiratory infections, but it has also been associated with vaccinations (specifically measles and chicken pox, both of which she had) AND insects bites. There's no recommended treatment, either. It usually lasts 4-6 weeks, but she could experience flare ups (including rash, achy legs, and loss of appetite) that may come and go for up to 6 months.

There are no known genetic causes (so Coco isn't more or less likely to get this) and no significant health issues to be concerned about in the future, except for this potential kidney damage. The dermatologist told me it was "quite rare" and then quoted "something like 10% of cases" which felt like a HUGE number to me, since I now face with the world with the understanding that the odds of anything bad happening to me or my family that are 1/160 or greater are basically guaranteed to occur.

My skewed logic aside, the doctors didn't seem too concerned about it since her appetite has improved and she hasn't had any sign of blood in her urine or anything like that. Still, we headed down to the lab to have blood work and a urinalysis.

I'd been to this lab at Children's hospital once before, when Coco was a teeny baby, and I felt the same way when I was there with her--terrified and also grateful. You see these parents there with kids who are visibly ill and although I haven't walked in those shoes, I can imagine the fear and the hope and the desire to learn everything you can but also to maybe protect yourself from knowing too much and being able to control so little.

Zuzu was a champ about her blood draw and made a really good effort to pee in a "hat" to collect her urine sample, but we ended up having to do it at home. My parents dropped off the specimen at the lab the next day.

Her blood tests have come back with everything in the normal range, and we see the pediatrician once more next week, at which time I hope we'll get the results of her urinalysis. The "rash" is already looking better, although her ankle was a bit swollen again Thursday night after she had a tumbling class.

I feel mostly relieved to have a clear diagnosis and thankful that it's not anything more serious. Of course, I've already run through the worst-case in my head (dialysis and kidney transplant), but it's been pretty easy not to dwell on that stuff because she feels/acts so normal 98% of the time. I'm now hyper aware of her complaints of a "stummy-ache," and she's catching on to that because she told me the other night that she had a "stumm-ache" and the only thing that would fix it was to eat chips for dinner.

Anyway, this HSP thing is also funny because most people have never heard of it, but I've also met three people who have known someone who had it (it mostly occurs in kids, but supposedly is more common in boys than girls). So I thought I'd put it out there (if you've made it all the way through this looooong post)--anyone know someone who has had HSP? My friend Erin's sister made a full recovery and had no further issues with it her whole life, so I hope that Zuzu is heading in that same direction.


  1. No HSP experience here, but I did get a million chigger bites on my legs the summer we lived in STL and it was one of the worst things ever. There were so many and they got all scabby and swollen and infected that I ended up getting antibiotics for them as well. They took FOREVER to heal. It sounds like Zuzu is being a real champ through all of it -- I was way whinier, I bet!

  2. Hi! Lurker pediatrician here. I've seen about a dozen cases of HSP, and none that progressed to kidney issues. Here's hoping you'll experience the same! I've been reading your blog for a few years now, and just moved to West County a couple of months ago. Haven't made it to the Magic House yet but I'm definitely planning to take my kids soon. BTW, I'm a big fan of Montessori schools; the availability of a Montessori elementary school was a big part of our move here.

    1. We should do more Montessori talk! I'm curious about whether the school you have in mind in the same one we are considering.

      I'm relieved to hear that your cases haven't been severe. I hope this is just a weird thing and not something we will have to continue to stress over!

  3. I wrote on your last post about rashes. My son also suffers from purpura and it is a reaction to vaccines. It is specifically listed on some of the vaccine inserts. The flu vaccine was the culprit for my son. There is also a genetic defect that up to 60-70% of the population has. It is called MTHFR. This makes children more susceptible to reacting to vaccines as their bodies are not able to detoxify. Most pediatricians are completely unaware of this as few take the time to read the lengthy inserts that accompany vaccines. My son's purpura flares about twice a year now. It looks awful and usually just covers his legs and behind. I shared this story on FB and had a woman from a small town reach out to me because she lost custody (temporarily) during an ugly custody battle because they thought his purpura bruises were abuse. My son has been to every specialist (including top pediatric rheumatologist at CHOC), but there and there's nothing that can be done. We are currently planning to go the homeopathic route.

    1. Whoa... Zuzu also had the flu vaccine, but I'd read that purpura is sometimes triggered by measles or chicken pox vaccines. I'm also going to ask more about this MTHFR genetic issue. That was the one anomaly on my blood work after a Eliza died that may have contributed to her death, though my OBGYN noted that it's actually a widespread generic mutation and has not been directly linked to stillbirth. I hate that there's nothing that can be done! From what I've read, having regular recurrences is rather unusual--have you found the same? Is your son bothered by the rash at all? Have you found any effective homeopathic treatments? I used lavender oil on Zuzu's legs and bottom because it's an anti-inflammatory, but I also read about some other oils. Her rash is fading fast, but I'll be on high alert to see if we have another incident.

    2. MTHFR is related to PreE and Hellp ...

  4. Sorry to hear she's not well. Hoping for a full & speedy recovery. xo

  5. Poor Zuzu! Glad you have a diagnosis but what a wild goose chase in the interim.

  6. Long time lurker here but I had to chime in....
    My oldest daughter had HSP when she was 2.5 (she is now 9.5). Hers presented as intermittent stomach ache/vomiting. She wasn't diagnosed until her 3rd emergency room visit and was then in the hospital for a full month because she couldn't eat. She had to have a PICC line and receive all her nutrition intravenously. She only had the leg rash on that third visit- before that her legs were clear. She required super high-dose steroids to get her intestinal inflammation under control, and then had to wean off the steroids over the course of almost a year.

    Clearly she had a highly unusual case but HSP can be a very serious autoimmune disease. She did not have any vaccinations in the months prior to getting sick and had been a healthy kid up to that point.

    We had her kidney function tested for the next 1.5 years and she never had any trouble- and she's now a healthy, happy almost 10-year-old girl. She has two younger siblings and neither has ever had any auto-immune issues (knock-on-wood).

    It's scary for sure but I'm glad it sounds like Zuzu has a fairly mild case. You did the right thing by continuing to seek treatment when the answers didn't seem right to you. I am sending you all quick healing vibes.

  7. Yikes!! Scary stuff, but I'm glad you finally have a diagnosis, and I hope Zuzu is back to normal soon. (((hugs)))

  8. I had HSP in 1992 until 1994. I'm Now 37 years old and telling my husband about this disease as I recently have had several kidney issues. It didn't occur to me that this was linked to my HSP experience that begin at 12 years old. I was considered a rare occurrence not only because I was outside of the age bracket, but because I was a female. My symptoms lasted for over 2 years and I was wheelchair-bound. I don't want to scare you, but I am now a Nurse and want to educate you on the seriousness of this disease and the little known facts that are out there. My HSP lasted so long I had University Physicians coming to Connecticut to visit me in the hospital to take blood samples back to their Laboratories. 23 years ago there was very little research, and it seems today there is not much more. To this day, my mother swears that my HSP was caused from mosquito bites. My abdominal pain was so severe but I had to have my stomach pumped, my joints ache so bad I couldn't walk comma and my purpura itch so bad I had to have nightly oatmeal baths. I was put on high doses of prednisone and every time I taper down to a lower dose comma everyone thought I was getting better and on the mend. But that I would be right back where I started with the severe abdominal pain and admitted to the hospital. After two years of battling this disease it suddenly went away and no long-term effects or associated with the disease. As I mentioned before comma it just came up recently when discussing sudden kidney issues with my husband. I came across your blog when doing my research on long-term effects of HSP in adults and kidney involvement and I just wanted you to know you are not alone, do not take any of your daughter's symptoms lately as HSP is debilitating and scary for a child and a parent. There's no textbooks or Physicians that will give you the right answers, only the people that have lived it.

  9. Hello, I stumbled across this article while doing a search for HSP and vaccinations. I am a 42 yo woman who had HSP as a 5 year old. I contracted it right after getting my kindergarten vaccinations, and after a bout with strep throat. It was 1981. The doctors had no idea. They gave me a spinal tap, which I will never forget, because they thought this was meningitis. Anyhow, I am just writing to say that at about 31 I started to have a lot of problems. I get kidney infections and kidney stones often. I have many many food intolerances, I have tons of digestive issues, and just like in 1981, in 2017, doctors still have NO IDEA. They make me out like I am crazy and feed me pain killers. Ive done extensive research on this. No doctor will ever tell you it happened due to vaccines. I also suffer from rheumatoid arthritis and lupus now...2 more autoimmune diseases. I don't have any answers, but I thought I would let you hear from an adult women who had it in the 80's. thank you for your article Valerie

    1. I'm so sorry to here this! Its scary to think my son could have this follow him with complications into adulthood ☹

  10. Hi Brooke,

    I am experiencing HSP right now with my son who is 2.5 years old. We live in Ontario, Canada. A few weeks ago my son had what seemed like a cold, really congested and a few spots around his mouth and torso. We took him to a walk on clinic but the doctor wasn't concerned. By the next day he had high fevers that subsided with tylenol but after 3 days of not going away I took him to our family doctor. Strep Throat. She started him on Amoxicillin. On Day 9 of his antibiotic he developed an all over rash. Doctor believed it was an allergic reaction to Penicillin. We stopped the meds right away. Two days later daycare called. He would not eat (unusual for our big eater) and wouldn't walk. That night, he still wouldn't walk. I thought the rash on his feet and toes was bothering him but by morning his left leg was swollen. I spent the day applying cold compress and calamine lotion, with tylenol for pain but by evening it was worse and he was inconsolable. It was Saturday night so we went to Emergency Room. The ER doctor was baffled so called in the Paediatric Doctor on shift, who told us about HSP. Bloodwork came back clear but there was a small amount of blood in his urine analysis. They admitted him to the Paediatric Ward. His rash had been disappearing on his arms and torso but was getting angrier on both legs and feet. We know now that he had 2 different rashes that overlapped which is why we didn't notice that his legs looked different. I learned that rashes cannot disappear only in one spot (tip to others). He had no fever throughout but also no appetite. Thankfully he drank a lot so they unhooked his IV after 3 days. Since there is no cure for HSP they sent us home once his leg swelling was down and he could place weight again. His treatment was only Advil, every 8 hours. He definitely perked after Advil and got bursts of energy for about an hour then would go downhill again. The day after being discharged, the HSP started showing on his arms and face. This time with some open sores. At our Family Doctor discharge appointment 2 days later, she sent us back to the hospital. So back to ER and readmitted to the Paediatric Ward, this time for 5 days. The open sore tests came back as Impetigo. He had an ultrasound on his testicles that were bruised and baseball sized but it all checked out as normal HSP swelling. While in hospital we learned that 2 other children in my son's daycare class had been in hospital, one on critical condition. Public Health put out a letter parents saying that the daycare had been exposed to Invasive Strep A Bacteria. General Strep A Bacteria causes Strep Throat and Impetigo, so I know where my son HSP was triggered. Of course, there's noway to know what caused the HSP but we we know what triggered it now.
    We came home yesterday from the hospital and he is doing well but I'm terrified to send him back to the place that triggered this in him!!!!
    How did you other parents handle sending your kids back to germ infested daycares and schools? We are considering not sending him back at all but not sure our alternatives.

    1. Wow those symptoms sound really intense and frightening (not to mention totally uncomfortable for your poor little guy). We actually think my daughter’s HSP was triggered by the bug bites she got, and her symptoms were much less severe. I did worry about her immune system and did homeopathic stuff—probiotics and essential oils, which at least made me feel like I was doing something. We did continue her enrollment in daycare. Our philosophy has always been that exposure to germs is inevitable. But I completely understand and sympathize with your impulse not to send him back! I hope you are able to find a path you’re comfortable with. My daughter has had no additional symptoms or adverse side effects in the past two years. I hope your little one will have a quick recovery!

  11. My 4 yo son currently has HSP. The rash is awful. He was diagnosed Day 2 of having the rash and severe swelling. Thank God his pediatrician knows her stuff. I was flipping out the night before when we took him to an urgent care, because everywhere else was closed. The doc there had no idea what was going on. She did xrays, because his ankles, knee and wrist were extremely swollen. She also did labs and strep test. Everything was normal. She put him on antibiotics and steroids just to cover the many possibilities. The next day at his peds office we received a correct diagnosis. We are currently on day 9 of this mess. It’s awful to watch. You feel completely helpless. You can only treat the symptoms and make them as comfortable as possible. I hate it. I hate seeing him hurt. But he finally has his appetite back. Yay. No vomiting, praise God. I don’t do well with vomiting at all!!!! Hopefully this will go away sooner than 4-6 weeks.

  12. My son also did not have any vaccinations prior to the HSP diagnosis. He did, however have some upper respiratory stuff going on. We though it was just allergies because it’s that time of year and his doc agreed. He didn’t have a fever, just coughing, sneezing and a runny nose... typical fall symptoms. Prayers to all of those whose kids have HSP. It will get better and thank god it’s something that will go away on its own and it not life threatening.