Henoch Schonlein Purpura

Overview

Henoch-Schönlein purpura (HSP) is now usually called IgA vasculitis. It is a condition where the smallest blood vessels in the body become inflamed. 'Vasculitis' simply means inflammation of blood vessels, and 'purpura' means purple or red spots on the skin caused by a little bleeding under the surface. Because these tiny vessels sit in the skin, joints, gut and kidneys, HSP can cause a rash, sore joints, tummy pain and, in some people, changes in how the kidneys work. It is not an infection, it is not contagious, and it is not caused by anything a child or adult did wrong.

Yes, in children it is the most common form of vasculitis. It is much less common in adults, but adults who do get it tend to have more serious symptoms, especially involving the kidneys.

It mostly affects children between about 3 and 10 years old, with the peak around 4 to 6 years. Boys are affected slightly more often than girls. Adults can get it too, but this is uncommon. It can happen at any time of year, though it is often seen in autumn and winter, following the seasons of coughs and colds.

Symptoms

Common symptoms

• A rash of raised, red or purple spots that does not turn white when you press on it — most often on the lower legs, ankles and bottom, and sometimes on the arms or trunk
• Painful, swollen joints — usually knees and ankles — which can make walking uncomfortable
• Tummy pain that comes and goes, sometimes with feeling sick or being sick
• Blood or protein in the urine, which you cannot see and which is only found on a urine test
• A mild fever and generally feeling tired or unwell
• In boys, swelling and pain in the scrotum (the pouch that holds the testicles)

Symptoms in children

• The rash is usually the first thing parents notice, often appearing suddenly on the legs and bottom
• Tummy pain can come on before the rash appears, which sometimes makes the early stage puzzling
• A child may refuse to walk or cry when standing because of sore, swollen knees or ankles
• Symptoms often start one to three weeks after a sore throat, cold or other minor infection
• Young children may simply seem irritable, off their food and more tired than usual

Symptoms in older adults

• HSP is uncommon in adults, but when it happens the rash can look different — sometimes with blistering, larger patches or small sores rather than fine spots
• Kidney involvement is more likely and tends to be more serious than in children
• Joint and tummy symptoms can be more intense, and recovery may take longer
• The rash may appear on the trunk and arms as well as the legs
• There is a higher chance of the condition coming back after it settles

Causes

Main causes

• HSP happens when a protein called IgA builds up in the smallest blood vessels and triggers inflammation. IgA is a normal part of the immune system that usually helps fight infection.
• In most cases it follows an infection a few weeks earlier — commonly a throat infection or a chest infection — which appears to set the immune system off in this way.
• Occasionally it has been linked to certain medicines or, rarely, to other triggers. Your doctor can review any medicines with you.
• It is not caused by food, by exercise, by stress, or by anything a parent or patient did or did not do. It is not infectious and cannot be passed to other people.

Risk factors

• Age between about 3 and 10 years — this is by far the biggest factor
• Being male, which makes it slightly more likely
• Having had a recent sore throat, cold, chest infection or other infection
• Having a family member who has had HSP — this is uncommon but has been reported
• Being an adult with HSP, which raises the chance of more serious kidney involvement

When to see a doctor

See a doctor urgently if:

• Any new rash of purple or red spots that does not fade when you press a glass against it
• Rash together with tummy pain, sore joints, or feeling generally unwell
• Blood in the urine, or urine that looks dark, cola-coloured or frothy
• Severe tummy pain, vomiting, or black stools — seek emergency care
• Sudden pain or swelling in the scrotum or testicle — seek emergency care
• Trouble breathing, or swelling of the face, lips or throat — call your local emergency number

Book a routine appointment if:

• A rash that keeps coming back, or spots that bruise easily without a clear reason
• Joint aches that linger after the rash has settled
• Follow-up urine and blood pressure checks, which are usually arranged for about six months after diagnosis
• Any concerns about your child's energy levels, appetite or growth after HSP
• Questions about returning to school, sport or work

Diagnosis

There is no single test for HSP. In most cases a doctor makes the diagnosis by examining the rash and asking about the pattern of symptoms — the combination of a typical rash, sore joints, tummy pain and a recent infection is usually enough. The doctor will also check blood pressure and test the urine, because the kidneys can be involved even when a person feels fine. In less clear cases, or when symptoms are unusual or severe, extra tests may be arranged.

Tests that may be done

• A urine dipstick test — a quick strip test that looks for blood and protein in the urine
• A urine test sent to the laboratory to measure how much protein is leaking
• Blood tests to check kidney function, blood counts and the immune protein IgA
• Blood pressure measurement, repeated at follow-up visits
• A stool (poo) test if there is tummy pain, to look for traces of blood
• An ultrasound scan of the tummy or scrotum if there is severe pain
• Rarely, a small skin sample (biopsy) or a kidney sample (kidney biopsy) if the diagnosis is uncertain or the kidneys are significantly affected

What to expect at your appointment

After diagnosis, the usual plan is regular check-ups — often weekly at first, then less often — with urine tests and blood pressure checks for at least six months. This is the most important part of follow-up. Most people need no other tests. If protein or blood shows up in the urine, your doctor may repeat the tests and refer you to a kidney specialist (a paediatrician or nephrologist) for advice. Bring a list of any medicines, and jot down symptoms and dates, as this helps the doctor see the pattern clearly.

Treatment

There is no specific cure for HSP, and in most children none is needed. The condition usually settles by itself over a few weeks. Treatment focuses on two things: making the person comfortable while the inflammation settles, and keeping a close eye on the kidneys so any change can be picked up and managed early.

Self-care at home

• Rest when tired, and let the body recover — but gentle movement and normal activity are fine as comfort allows
• Drink plenty of fluids, especially if there is fever or vomiting
• Use a simple pain reliever if needed, after checking with a pharmacist or doctor about which one is suitable — some painkillers are avoided if there is any concern about the kidneys
• Ease sore joints with rest, support and gentle elevation; a cool pack wrapped in a cloth can help
• Wear loose, comfortable clothing over the rash and avoid very hot baths or tight waistbands
• Keep a simple diary of symptoms, urine colour changes and temperature to share at appointments
• Attend every follow-up urine and blood pressure check, even when everyone feels completely well
• Keep the child home from school while they feel unwell and have a fever, then return as energy improves

Medical treatments

Most people need only symptom relief and monitoring. If tummy pain is severe or the kidneys show signs of inflammation, doctors may prescribe a short course of steroid medicine to calm the inflammation. For more serious kidney involvement, a kidney specialist may use stronger medicines that dampen the immune system, along with medicines to control blood pressure and reduce protein leaking into the urine. Which approach is used depends on the person's age, symptoms, test results and general health, and it is always decided with a specialist. Antibiotics are not used unless there is a separate bacterial infection. Your doctor or pharmacist can explain any medicine, why it is being suggested, and what to watch for.

When is surgery considered?

Surgery is rarely needed in HSP. It may be considered if the bowel becomes blocked or folded on itself (a condition called intussusception), or if there is a genuine concern about twisting of a testicle. These are uncommon and are checked for with an ultrasound scan if symptoms suggest them.

Living with this condition

For most families, HSP means a few unsettled weeks: a child who is sore, tired and off school, plus a run of appointments for urine and blood pressure checks. The rash can look alarming but usually fades gradually, sometimes leaving faint brown marks that disappear over time. Symptoms often come in waves, so a good day followed by a worse one is normal. Keep a note of test dates and results so you can see progress, and ask your doctor for a clear written follow-up plan before you leave the appointment.

Lifestyle tips

• Build in extra rest while symptoms are active, and return to normal routines gradually
• Keep up fluids and regular small meals if the tummy has been sore
• Avoid contact sports while joints are painful or swollen, then ease back in
• Take simple steps to reduce the chance of everyday infections, such as handwashing
• Keep a folder or phone note of appointment dates, urine results and blood pressure readings
• Tell any new doctor, dentist or pharmacist that HSP has been diagnosed, especially before any new medicine is started

Diet and exercise

There is no special diet for HSP. A normal, balanced diet with plenty of fluids is best, and small frequent meals may be easier if the tummy has been sore. If vomiting is a problem, offer fluids in small sips often. Gentle everyday activity is encouraged — walking, playing quietly, and returning to PE or sport once joint pain has settled. There is no need to stay in bed once the fever has gone and energy is returning. Build back up slowly over a week or two rather than going straight back to full training.

Mental health and emotional wellbeing

A visible rash, a limping child and talk of kidney tests can be frightening, and it is very common for parents and patients to feel anxious, especially in the first weeks. Children often pick up on that worry, so simple, honest explanations help: 'your blood vessels are a bit cross, and they are healing'. Sleep can be disrupted by joint pain, which makes everything feel harder. Talk to your doctor about any worries — no question is too small. If you or your child ever feel overwhelmed, hopeless, or have thoughts of harming yourselves, contact your local emergency number or a crisis helpline in your country straight away, and reach out to a trusted adult, friend or your doctor.

Prevention

HSP cannot be prevented. There is no known way to stop it happening, and nothing you did caused it. It is not contagious, so no one needs to be kept away from others. Because it often follows an ordinary infection, the only sensible general advice is the usual: handwashing, staying up to date with routine vaccinations, and getting enough rest. If HSP has already happened, keeping every follow-up urine and blood pressure appointment is the most useful thing you can do to protect long-term health.

Vaccines

There is no vaccine for HSP. Routine childhood and adult vaccinations are not known to cause HSP, and there is no reason to avoid them after HSP. If you have any questions about timing, particularly if the person is unwell or on immune-suppressing treatment, ask your doctor or nurse.

Screening programmes

There is no screening programme for HSP. Instead, anyone diagnosed with it is offered planned follow-up: urine tests and blood pressure checks for at least six months, and sometimes longer if there were any kidney changes. If you have had HSP in the past and are now being seen for something else, do mention it — your doctor may want to check your blood pressure and urine.

Complications

If left untreated

• Kidney inflammation (called IgA nephritis) — this is the most important complication and can lead to blood or protein in the urine, and rarely to longer-term kidney problems
• High blood pressure, which can develop quietly and is why blood pressure is checked at every visit
• Bowel problems, including bleeding, or rarely a blocked or folded bowel
• Twisting of a testicle in boys, which is a surgical emergency
• The rash or joints flaring up again — recurrences happen in about a third of people but are usually milder
• Tiredness and time off school or work during the active phase

Long-term outlook

The outlook is good for most people. Around 9 in 10 children recover fully within four to six weeks, and many have no further trouble at all. Some children have a second bout of rash or joint pain, usually milder than the first. A small number — roughly 1 in 20 children — have some lasting kidney change, and this is more likely in adults. That sounds worrying, but the reason for the six months of urine checks is exactly this: changes are usually picked up early, when they can be monitored closely and treated if needed. Most people with HSP go on to live completely normal, active lives.

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