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Lumpy, Bumpy, Scary Rash: HSP

August 10, 2010

Palpable purpura of HSP; courtesy of okwikikim at the wikipedia project.

Suppose your child developed this rash; how long would you wait before seeing the doctor?

We doctors describe this dramatic rash as “palpable purpura.” Palpable means they are raised; we can feel them when we stroke the skin. Purpura refers to blood outside of vessels. If you press down on the lesions, they do not blanch. Smaller, flat lesions without blanching would be called “petechiae” in doctor-speak. Several conditions can cause this sort of rash. The most problematic, meningococcemia, is a bacterial infection that can kill a previously healthy child in a few hours; we now have a vaccine for this condition that I jabbed into my kids as soon as its approval came through. Scary stuff.

The child in the photo has a less threatening illness, Henoch-Schonlein purpura. HSP is a form of vasculitis or inflammation of the blood vessels. The blood vessels become leaky, allowing blood to escape into the surrounding tissues. Essentially, affected children get bruises all over the place! This process can happen in any organ in the body. Some areas are more commonly involved:

  • Skin: As blood leaks into the skin it produces a bruise-like rash called petechiae (when pinpoint in size) or purpura (when larger in size). This rash is often lumpy.
  • Joints: Vasculitis in the joint can lead to swelling and pain (arthritis) or pain without swelling (arthralgias).
  • Gut: Bleeding into the walls of the stomach and intestines can produce nausea, vomiting, abdominal pain, diarrhea, or constipation. Blood in the vomit or stools is not unusual.
  • Kidney: Vasculitis in the kidney is given the name glomerulonephritis. Glomeruli are the small tufts of blood vessels that filter our blood to make urine. When inflamed, there may be leaking of blood into the urine (hematuria) and protein into the urine (proteinuria). In severe cases of nephritis, blood pressure may be increased (hypertension), proteinuria may be heavy enough to cause swelling (nephrotic syndrome), or the kidney’s ability to eliminate wastes may be compromised (renal insufficiency or failure).

Other organ systems may be rarely involved. These include the eye, brain, lung, and liver.

The cause of HSP is unknown. Why one person gets it worse than another is also unknown. Seems we don’t know a whole lot of things about HSP!

There is no specific test for HSP. Your doctor makes the diagnosis based on typical findings described above. Often other forms of vasculitis may be ruled out with blood tests or, in extreme cases, a biopsy of the skin. HSP vasculitis looks different from other types under the microscope. Even if there is no evidence of involvement of the kidney, blood and urine tests are usually obtained to look for this complication.

With severe or unusual involvement of any organ system, more tests may be needed. Often a specialist is consulted. For severe renal involvement, a kidney biopsy may be necessary to confirm the diagnosis as HSP and guide further treatment.

Most cases of HSP produce uncomfortable but non-life-threatening problems that come and go for up to 6 months with no specific treatment. Acetaminophen (Tylenol(R)) or other medicines may be used for pain control, but HSP usually goes away on its own.

Long-term problems from HSP are most often caused by bad glomerulonephritis. Treatment for this often includes intravenous and oral steroids (drugs such as prednisone, not the muscle-building steroids) and chemotherapy drugs such as cyclophosphamide. These treatments can  stop the inflammation of the blood vessels and prevent permanent kidney failure from developing. Control of high blood pressure is also very important in preventing kidney failure.

The NIH now has a nice page with more information about HSP.

  1. That’s fucken horrible!

    • HSP looks horrible, but it generally is self-limited with no long-term consequences. You don’t want to be one of the unlucky handful of kids who get severe glomerulonephritis, but most other children are well in a few weeks.

  2. I’d wait about 3 seconds between seeing this on my child and heading toward the ER. That’s purely a layman’s take.

    I’m not a completely naive layman… if the ER doc told me this was self-limiting, I’d believe it. But… what kind of parent would NOT get that checked out?

  3. Camilla permalink

    I went to 4 different doctors with this rash and none of them diagnosed me properly it wasnt until i went to one particular GP and within 5 minutes she diagnosed me. I am so angry that i have got this because its itchy hot and sore and i will have it for weeks. Why isnt there a cure!!!

  4. Melissa permalink

    My son has had the rash for 4wks. was healing and he was feeling good in himself. Then over
    night has come up again, with the internal pains are happening again. Not nice at all. You would
    think there would be a cure!!

    • Unfortunately agencies don’t grant a lot of research funding to diseases that make you miserable but resolve. We still don’t kno w what causes HSP!

  5. Helen McNeill permalink

    My little 6 year old girl developed this rash, it is awful!! She has been sick quite a few times and is now slowly getting back to normal. The rashes look so sore and the bruises look awful, we ended up in our local A&E after our GP referred us straight away, it was really worrying, she has had the rash nearly two weeks now and is not showing any signs of clearing up. The rash stops at her knees and is just on her right arm. Wish there was some cream to put on but the consultant said there is nothing for it. The community nurse has to come out every other day now for the next three weeks to test her urine and under the hospital now for the next six months.

  6. melanie permalink

    My son, who is 7, was just diagnosed with HSP. I’m really freaking out. We thought he had the flu, because his tummy was cramping and he threw up. I just found out today.

    His legs are so sore, he can hardly walk. He feels rotten, and It’s killing me. He had strep throat, and now this struck – very suddenly.

    The rash looks very scary, especially for a 7 year old. I’m very scared about what I’ve read about kidney problems and bowel inflammation. I read It’s very painful, and that’s very upsetting.

    • Helen permalink

      My daughter is just getting over HSP, it was horrendous, she had the rash alover her legs, arms and bum, you couldn’t put a pin between the rash and the bruising was awful. She is 6 yrs old. It is worrying, but she is getting up every morning and they seem to be disappearing over night. The nurse has been out every week to monitor her BP and urine but she has been fine. Every day she is getting better. The hospital told me it is very common, but I don’t know anyone who has ever heard of it!!!

    • How is your daughter now? 😦
      My son is 7 & was just diagnosed with this disease. My husband and I are really scared for him. He had the rash very severe all over his legs, bum, arms and a few on his lips now! He has had swollen knees and hadn’t been able to walk. We have been in the er 3 times in the past 48 hrs. The tummy aches have begun tonight and I feel so bad that there is nothing I can do to help him!! 😦

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