Henoch-Schonlein Purpura aka HSP

My neighbors’ 9-year-old daughter was recently diagnosed with a condition called Henoch-Schonlein purpura. This apparently followed a viral infection. She is under the care of a physician and this was diagnosed by a biopsy. I have done several searches “on line” and have not come up with any information. Would you be able to shed some light on this subject or steer me in the right direction as far as resources? What is the cause of this; implications – long term?
A Registered Nurse – Froedtert Memorial Lutheran Hospital – Milwaukee, Wisconsin

Henoch-Schonlein Purpura

Dr. Greene’s Answer:

No one knows what causes it. We do know that it often follows a viral respiratory infection. It seems to be some kind of allergic reaction to the virus. It has also been seen following Strep throat, prescription medicines, bee stings, chemical toxins, cold exposure, and food allergies. It can occur in epidemics. In the Northern Hemisphere it occurs most commonly between November and February. It is often accompanied by a low-grade fever, and just not feeling well. It most often affects children ages 2 through 10, boys more often than girls. The older the child (or adult), the more likely it is to be serious. We know that it can be life-threatening, but that most children recover. “It” is Henoch-Schonlein purpura (HSP).

HSP is a type of vasculitis — an inflammation of blood vessels — that was named for Drs. Henoch and Schonlein, who each discovered it independently more than 30 years after it was discovered by Dr. Heberden, who got no credit.

Tiny blood vessels in the skin swell and then hemorrhage, giving rise to the characteristic rash of HSP. The rash usually begins with small hives, or red patches, or red bumps, which can appear anywhere on the body, but especially on the legs and buttocks. This rash represents swollen blood vessels, and the spots blanch with pressure, since pressure moves the blood along the vessel. The allergic nature of the rash often makes it itch. As time passes, blood leaks from the swollen vessels, the rash changes from red to a bruised, purple color (hence the name purpura), and the rash no longer blanches when pressed. As the tiny bruises heal they turn to a rust color, and then fade. Each spot lasts for about five days. Often the rash comes in several crops, and a single child may have a rash of a variety of different colors. All children with HSP have the skin rash (by definition). The rash resolves without trace. (“A savvy mom named Beth Anderson wrote and suggested that when her son had HSP, “the term “blood blisters” was the key to describing the rash for those who couldn’t see it in person. Most people have had a blood blister and can relate to the term better than bruises.”)

The skin rash is the most obvious, and most common, finding in HSP — but by no means the most serious. Blood vessels in other parts of the body can also be involved –most frequently in the joints, the intestines, and the kidneys. Rarely, vessels in the muscles, eyes, testicles, lungs, heart, and brain have become inflamed, sometimes leading to serious consequences.

Joint involvement occurs in two thirds of the children. The knees and ankles, particularly, often become swollen, tender, and painful with movement. This arthritis can be quite debilitating, but usually resolves in just a few days. No permanent deformity results, even with the most severe arthritis.

Inflammation of the blood vessels of the GI tract occurs in more than half of the children. Most of these experience abdominal pain, often quite severe. They will often vomit — sometimes vomiting blood. More than half of the children with HSP will have bloody stools. Serious short-term complications most often come from the GI involvement, some of which require observation in the hospital and some require surgery.

Kidney involvement affects between one-fourth and one-half of the children with HSP. They may have blood and/or protein in the urine. They may even have kidney failure. Serious long-term complications most often come from the kidney involvement.

Full-blown HSP may appear suddenly, or different symptoms may appear gradually over several weeks. The order in which the symptoms appear varies. A child might have unexplained abdominal pain, or knee pain, or bloody stools, or blood in the urine for a week before other symptoms appear to bring the correct diagnosis into focus. The diagnosis is usually made when the rash turns purple.

Most children recover fully. The disease may be quite mild and last only 2 or 3 days. For those with moderate to severe symptoms, though, the disease is usually gone within a month. Two-thirds will never have a relapse. For those who do, it is usually within the first four months particularly after the child gets another cold or is re-exposed to the offending agent. Thankfully, the relapses usually are both milder and shorter than the original episode.

Rarely, children die from complications experienced near the time of diagnosis (bowel perforation, hemorrhage, seizure, stroke, etc.). Having made it through the acute crisis, the long-term outcome depends on the extent of kidney involvement. With no kidney involvement, full recovery is the rule. One-fourth of the children with kidney problems will still have detectable problems years later. By 8 years after onset, however, only 2% will still have any kidney problems. The long-term prognosis can often be determined from a renal biopsy early on.

There is no specific treatment for HSP. If the initiating trigger is identified for an individual child, everything possible should be done to remove it. If the cause is thought to be a bacterial infection, such as Strep throat, prophylactic antibiotics are often given once the infection is eliminated, to prevent recurrence. Anti-inflammatory drugs, and sometimes immunosuppresive drugs, are used to provide much-needed symptomatic relief. Steroids are controversial – they may cause dramatic reversal of GI or brain involvement, but have not been shown to be very effective for the kidneys.

. HSP is a very serious disease. Thankfully, it is also very uncommon. The odds are that your dear little friend will recover from this with nothing to show for it except some bad memories.

Dr. Alan Greene

Dr. Greene is the founder of DrGreene.com (cited by the AMA as “the pioneer physician Web site”), a practicing pediatrician, father of four, & author of Raising Baby Green & Feeding Baby Green. He appears frequently in the media including such venues as the The New York Times, the TODAY Show, Good Morning America, & the Dr. Oz Show.

  1. Machelle

    Hi – My son’s onset of HSP was back in September that occurred from getting strep throat. He was in the hospital for 2 days because of dehydration from vomiting so often and not being able to keep anything down and missed 2 weeks of school. We went to 2 doctors and 2 hospitals until finally the admitting ER doctor instantly knew based off the symptoms (after waiting the night before for 4 hours because the admitting nurses didn’t feel that he was a priority!! We went back at 6 am and explained our frustration and luckily they got us right in). After leaving the hospital, he has had mild episodes of the rash, stomach pain, and headaches. We were directed to the children’s hospital and now in the care of a Ped. Specialist. We are down to monthly urine tests (blood still present in the urine) and blood pressure tests twice a week (another side effect of his HSP). I fear I have strep throat. Could this cause another outbreak of HSP if my son gets my strep throat?

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    • Machelle,

      I’m not a physician, but I can tell you Strep is an interesting infection that we are learning more about all the time. With your son’s history, it’s wise to be vigilant and not wait to see a doctor if you suspect he might have strep throat.

      Best,
      @MsGreene

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  2. Lisa

    I am mid 40’s and have been diagnosed with IGA Nyphrofothy when I was a child. I think I had this a month ago. I started with stomach cramping, then the bottom of my feet itched terribly and then the (what looked like hives) rash started on my feet. Then moved up my legs. Would this be consider as symptoms of HSP. The one thing that bothered me the most was the bottom of my feet itching. It itched so bad I could not stand it. My feet are very ticklish and maybe that’s why I thought it to be weird because when I scratched the bottom of my feet it did not tickle it was a relief. I have no symptoms now.

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  3. Andrea

    Can a child that had HSP when he was 6 get it again when he’s an adult. My son is 19 now but I think he’s showing signs of it again. He was admitted to hospital for 2 nights with a rash sore throat couldn’t stand the light stiff neck. Was sent home after 2 days with antibiotics for his throat. Now he has come out in a rash but all different arms back of wrists, sides and spine also on the back of his knees 4 circles that look like they are going purple. He also has a low temperature 35.4.

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  4. Jennifer

    My 6 year old daughter was diagnosed with HSP 14 days ago. The rash, stomach pain and arthritis comes and goes and each day is unpredictable. Her urine analysis has been clear and a blood panel taken early on showed no issues.

    Our pediatrician feels confident that our daughter’s case is “text book” and she will be fine but I am concerned. The rash has now spread from her lower extremities to her arms and hands. And, a new batch of the rash has appeared on her legs and bottom and the pain & swelling are still present. This round of rash is itchy while the first 2 weeks it was not.

    Is there any research about specific food allergies triggering HSP?

    Thank you!

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  5. Sinead

    Hi
    My son was diagnosed with hsp a year and a half ago after a viral infection his urine is tested once a month with small traces of blood in 1 of 4 tests but he is still having outbreaks of the rash which are getting more severe. He is also suffering with severe joint pain. He also suffers constipation because of this. Can you tell me what are the next steps we should take as I don’t think all the painkillers are good for someone so young

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  6. hemalatha

    Hi,
    My son had hsp with red spots on the legs and had got rid of them in 2 to 3 weeks. Once in a year I did urine examination for any protein or blood. Tests showed none of them.
    But today I saw some blood in the stool, is it related to hsp or normal…please reply wih suggestion..is it possible to have any kidney or stomach involvement after 2 years.

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  7. Jen

    My son, aged 8.5 was diagnosed with HSP after a dose of Flagyl (doctor prescribed after thinking he had a gastro issue) had me running back to the doctor with stomach pains and a rash. I thought he must have been allergic…

    Initial dose of prednisone stopped the stomach pain and achy joints but then a few weeks later the rash came back (albeit lighter) and stomach pain for a day.
    His urinalysis showed blood and protein consistently and last week we endured a renal biopsy. He is a happy chappy who to all purposes looks hail and hearty, but he has been asked to return to the hospital next week to commence IV steroids. I am concerned what this means. What are the side effects, what is the long term prognosis? What does IV steroids involve?

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  8. Arti

    Hello,
    My son had FLU with cold and congestion in December and after that in January I saw a small rash near his top eyelid. Slowly the rash spreaded on both the eyes, and is there since then. It has been 4 months and I still see the black rash around his eyes shattered. Not sure if this is HSP. I took him to ENT specialist where she did is scan which showed his sinuses are fine. I also showed it to our pediatritian and she does not know what it can be. She referred us to a dermatologist by saying that it looks like a rash ruling out the possibility of lack of sleep or sinus infection.
    If anyone has experienced similar or knows about something similar please respond.

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  9. Elissa S.

    Hi Dr. Greene,
    I was diagnosed at 2yrs old with HSP. My case was severe and required surgical removal of a section of my intestines. I am now 43, and I have a list of health issues that seem to stem from my HSP. I was wondering if you could recommend some resources where I might find more information on long term/life long effects of HSP. All the research I have found says doctors do not really know of long term effects except renal issues, but I am pretty sure I can connect all my health issues to the damage of my HSP. Any suggestions of where to look or even a specialist that might handle HSP cases? I would really appreciate any information you could give me.
    Thank you,
    Elissa

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  10. Maureen

    I’m wondering if people with HSP should stay out of the sun.

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  11. Manjula

    Recently we found that my son has hSP(one week back).

    He has been suffering from severe stomach pain and blood rashes. I am so worried about him. I don’t know the reason, we went to pediatrician, should we consult any other doctors or dermatologist?

    We don’t know what to do, no medication nothing.

    Any suggestions please…

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    • wendy smith

      I’d keep a close eye on him and if worried consult your local doctors or hospital.

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  12. Maureen

    My daughter, 13, was diagnosed with HSP on Feb. 3rd. Previous to that, she had the flu and a respiratory infection and was given an antibiotic. The rash showed up first on her palms. They were like little cuts covering the whole palm on both hands. Later that day, her legs, ankles and butt were covered as well. It kept coming and coming. The rash on her legs lasted about 2 weeks, with just a handful here and there on her legs and ankles. The rash on her hands still come and go.

    In addition to the rash, she had severe joint pain in her knees, ankles, elbows, and hip, as well as nausea, headaches and abdominal pain. The joint pain is better but she does still experience it frequently in her knees and ankles mostly. She is still having headaches too. Nausea is the biggest complaint now. It’s been two months and she is still miserable. She hasn’t done a full day of school for over two months. At this point, the rash keeps showing up in her hands and it bothers her to hold a pencil!

    I feel awful for her and wish I could help her. Ibuprofen and Tylenol does not help but she does sometimes get some relief from Alleve. Sometimes I wonder if a lot of it is nerves or her reacting to the stress of it. If I mention this, she thinks that I think she’s faking. I know she’s frustrated. I’m frustrated too. The nurses at her school are losing patience with her showing up there every day even though we have a doctor’s note. They don’t get it. When is this going to go away??

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    • wendy smith

      Hi I read with interest your story. I’ve not come across a story of people with these symptoms on the hands. Sorry I am unable to provide any further answers. I hope your daughter is well soon

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    • corrie

      My son has HSP. He got small blisters on his hands and they swelled up. His rash begins as what looks like bug bites and they itch. Then it begins to look more like what they describe the HSP rash to look like. He’s had it for 6 weeks now. For three weeks he couldn’t walk bc his joint pain was so severe.

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  13. David Norman

    Hi Everyone,

    We are into day 4 after my son aged 9 has been diagnosed with HSP, learning fast about what this means for him.

    The week before seeing the GP my lad had a cold and the day before seeing the GP, complained of bruises on his forehead.

    Following that we are are now on day 4 and he has difficulty in getting around. Such a shock as he was only playing in his football team the other week.

    After discovering this we just await for the outcome over many weeks I guess. Regular checkups at the doctors have been arranged.

    Wish the best for all.

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    • natalie

      My son is the same age and has hsp also plays sports, but I haven’t let him play right now. I was curious to know if your children have been able to go back to their sport.

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  14. Jacques

    I have HSP and its going on more than 2 years now. I only take Dapsone as prescribed by my doctors. I have done many, many tests and nothing else internally (organs) have been affected, although I do have occasional severe joint pains. The doctors have assured me that the disease after all this time will basically stay with my skin and not affect any organs. If I stop taking my dapsone, I flare up within 2-3 days. I feel like the meds are only hiding the illness and I will run into severe health problems one day. I am 52 years old.

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  15. sandra brooker

    Hi my 4 and a half year old has HSP. He had the rash and spots and more spots and rashes. It seem to appear more on him on a daily basis. Can you tell me if this is right? His name is Anthony and I love him dearly.

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  16. E. Low

    Can the HSP involving the testicals cause sterility?

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  17. Al Martia

    I’m an adult (40) who had HSP 6 months ago; it went away any never came back. My doctor ses to think I was allergic to an antibiotic (ciproflaxin?) that I was taking for a chest cold. The HSP didn’t occur until I took that medicine.
    My question is, will I have a recurrence if I get another cold? or should I be ok as long as I stay away from that family of antibiotics? I’ve had every blood test under the sun for the past 6 months and I was told my blood is “stable” and within normal range. I’m scard to get a cold thinking that this will be the norm every time.

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    • Kristen

      I also had HSP after taking an antibiotic, as a child, and again as a teen. Then in my 30’s I took a different antibiotic and had it again! I do not get recurrence when ill, just when I take certain antibiotic meds.

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    • wendy smith

      Hi, It is normal to monitor the blood after hsp. I’d be inclined to keep a record of your health. You can never say when it might flare up. Each case is different. However, its always worth while keeping a close eye on things including fatigue, joint pain, urine problems, etc.

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  18. candybowman90@gmail.com

    My 8 year old daughter got HSP August 2012. She has already went into complete renal failure. She is on dialysis 3 times a week which they started a week ago. The doctors say she is not a candidate for a transplant until the HSP stops. Be honest with me pleader.. what are her chances is surviving this.

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  19. sue

    hi my daughters 9yrs old and has had it 2yrs and still on going,

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  20. elise beer

    hey I had this when I was I child is there any long term problems that come out later in life. as an adult. I am having some interal problems. and just want to now if this can be a reselt of having hsp as a kid

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    • hey elise! when it comes to long term problems from HSP, the big one we know about is kidney problems. As I said in the article, somewhere between 25%-50% or so of the people with HSP have kidney problems in the short term. Of these, most do not have long term problems, but some do, years later as an adult, even after having recovered fully in between. Apart from kidney problems, though, I’m not aware of other proven internal issues later as an adult. All my best in sorting out what is going on!

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      • elise beer

        thanks ive been having lots of heart burn and been goin to the bathroom to much thought maybe but that’s ok ill look at other reason

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    • Elissa S.

      Hi Elise, my name is Elissa and I had HSP when I was 2yrs old, I’m now 43. I found your post while looking for answers to certain health issues I have that seem to lead back to my HSP. My case was severe and I had to have surgery to remove a section of intestines. Since childhood, EVERY time I eat, I am in the restroom within 10 minutes. I have been diagnosed with malabsorption issues, CHF, iron, vitaminD and B12 deficiencies among other issues. I have had a VERY difficult time finding any information on long term/life long effects of HSP, but am desperately looking for answers! Would love to compare notes with someone who may have some of the same issues I have experienced. If you would be interested please let me know and maybe we can chat by email or how ever you might be comfortable doing so. I hope you find the answers you are looking for, and know that you are not alone in your search for answers.
      Best Wishes,
      Elissa

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      • Charlotte

        Hi my son 5 years was diagnosed with hsp August 2014. Although the swelling and rash have gone, (ended up Jin a wheelchair for a few weeks) he still has days where he limps or is uncomfortable, 10 months on. I think the research needs to be redone as they say it usually goes away in a couple of months.

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