Henoch Schönlein Purpura (HSP)


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Fast Facts

  • HSP causes a rash called purpura, which is a red to dark purple rash caused by inflammation of the blood vessels.
  • Other symptoms include painful swelling around the joints and abdominal pain.
  • Children with HSP should be monitored for development of kidney disease.
  • Most children with HSP recover fully and many require no medications.
  • Up to one-third of children with HSP can experience multiple episodes of rash.

Henoch Schönlein Purpura (HSP) is a type of vasculitis, which means inflammation of the blood vessels. The classic symptom of HSP is a red to dark purple rash, called “purpura”, which is often most severe on the legs and buttocks. Other symptoms include painful swelling around the joints and abdominal pain. Many children with HSP recover completely, but up to one-third of patients can develop kidney disease. Treatment depends on the individual child’s symptoms. Monitoring for kidney disease plays an important role in early detection and treatment if this complication happens.

In North America, HSP is the most common form of vasculitis in children. It is most common in ages 3-15 years and is more common in boys than girls. It rarely occurs in adults. HSP can occur at any time of the year, though it is more common in the winter.

What causes HSP?

The exact cause of HSP is unknown. In some patients, the symptoms start after an upper respiratory tract infection or cold, but HSP is not contagious. In patients with HSP, the person’s own immune system attacks blood vessels in the skin, intestines, joints, and kidneys. Inflammation in the blood vessel wall leads to bleeding into the skin, which causes the rash of HSP. Bleeding into the stool and urine can also occur.

What are the signs and symptoms?

  • Purpura is a red or dark purple rash that does not fade away when pressure is applied to the skin. Sometimes this starts as tiny/pinpoint dots called petechiae or as a red rash that darkens with time. The classic description is “palpable purpura”, meaning that the rash is raised and can be felt. It is usually effects the legs and buttocks. In some cases, large blisters can develop in the areas of the rash.
  • Pain and swelling around the joints usually resolves within a few days to a week.
  • Swelling under the skin may occur anywhere in the body, but most often in the hands, feet, face and scrotum.
  • Abdominal pain often surrounds the belly button and may be “colicky” (crampy pain that comes and goes). Rarely, children can experience bleeding from the bowels, or a serious condition called intussusception, in which one section of the bowel to telescope inside the next. Intussusception is an emergency and can require surgery.
  • Kidney disease can range from blood or protein in the urine with normal kidney function to more severe kidney injury causing swelling in the legs. Often, kidney disease is only detectable through laboratory analysis of the urine because the patients do not feel any symptoms. Usually these kidney abnormalities resolve, but rarely they can become chronic and cause more serious kidney problems. Kidney failure is rare.

How is HSP diagnosed?

There is no specific test to diagnose HSP. It is diagnosed based on recognition of the classic symptoms, and exclusion of other conditions that can cause a similar rash. In many children with a classic rash, minimal testing is needed to establish a diagnosis of HSP. The rash is necessary for the diagnosis of HSP but is not always the first symptom to appear. When joint pain, swelling, or abdominal pain start before appearance of the rash, diagnosis can be challenging.

Tests in children with suspected HSP depend on the patient, but might include the following:

  1. Platelet count and coagulation studies to looks for other causes of bleeding.
  2. Laboratory tests to rule out other causes of vasculitis.
  3. Evaluation of kidney function by blood pressure check, creatinine level, electrolytes, and urine sample.
  4. In some patients, a biopsy may be taken of the skin, kidney or other tissue. Biopsies in patients with HSP often show high levels of a specific type of immune protein, called immunoglobulin A (IgA).
  5. Imaging of the bowels may be performed if abdominal pain is severe.

How is HSP treated?

Most children with HSP do not require any specific treatment and recover with time alone. Joint pain can often be controlled with rest and over-the-counter medications such as acetaminophen, ibuprofen, or naproxen. Steroids given by mouth or through an IV infusion are typically given for severe abdominal pain. Your doctor can discuss if steroids would be necessary in your child.

Many children with HSP can be managed by their primary care provider (a general pediatrician or family practitioner). If symptoms are severe or additional expertise is needed, a rheumatologist can help evaluate and treat a child for HSP.

One of the most important parts of HSP management is monitoring for kidney disease. Temporary blood or protein in the urine is common. Children with worsening urinary findings like high blood pressure or rising creatinine (a blood marker of kidney function) should be seen by a nephrologist (kidney doctor). A biopsy may be needed to assess for kidney disease. Some children with kidney involvement will need treatment with long-term immune-suppressive medications to prevent kidney failure.

Living with HSP

Most children with HSP have resolution of symptoms within a few weeks to months and recover completely. Even though HSP is usually a short-lived illness, having HSP can be stressful for children and families. The appearance of the rash can be distressing to a child. Some children experience joint or abdominal pain intense enough to require admission to the hospital.

In the first weeks of HSP, activities may be limited by joint or abdominal pain. Once these symptoms improve, children can go back to enjoying school and the activities that they love. If your child returns to school while the HSP rash is still visible, the school and teachers should know that the rash is not contagious.

After HSP symptoms resolve, up to one-third of children have a recurrence, usually within the first few months. It is important for families to know recurrences are common, especially of the rash. Recurrences are often milder and shorter than the first episode.

Urine screening and monitoring of the blood pressure is recommended for at least six months after the initial diagnosis, so it is important to follow the screening instructions provided by your doctor. Monitoring should happen weekly for at least six weeks after the diagnosis. Early detection of kidney problems is important to treat and prevent chronic kidney problems.

Written March 2019 by the Pediatric Rheumatology Special Committee, and reviewed by the American College of Rheumatology Communications and Marketing Committee.

This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical or health condition.

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