My Child is Diagnosed With Henoch Schonlein Purpura(HSP) – What Should I Know by Dr. Sagar Bhattad

Henoch Schonlein Purpura is a disorder or a condition that causes inflammation of the small blood vessels. The inflammation is more common around the legs and buttocks, causing a red or purple rash in the affected areas.

Henoch Schonlein Purpura (HSP) is also known as IgA vasculitis. It can affect the skin, joints, sometimes the intestines and in rare cases, even the kidneys. Though more common among children below the age of 10, HSP is also seen in adults. In the case of adults with HSP, the symptoms are more severe.

HSP usually occurs as an after effect of an upper respiratory infection. It is not contagious, so it cannot pass on or spread to others around your child. Your child might feel better on their own after a few days. However, they need to be under constant observation to ensure the disease does not spread to other parts of the body or affect the kidneys.

Signs and Symptoms of Henoch Schonlein Purpura

Henoch Schonlein Purpura has distinct symptoms which cannot be confused with other diseases or infections. Some of the common symptoms that can indicate your child may in fact have HSP are:

• Rashes on the skin, especially on the lower half of the body. These small red or purple spots that occur due to the swelling of the blood vessels are called Purpura. Sometimes the Purpura can be seen even on the arms, trunk, or face.
• Bruising in some cases
• Swelling or pain in the elbows, ankles, or knees. These pains usually occur before the onset of the rashes and will subside as the disease clears out. Generally, there is no lasting impact of these pains and swelling.
• Many children complain of stomach aches along with vomiting. This is one of the classic symptoms but is not a must in every case diagnosed with HSP.
• If HSP affects the kidneys, you can find small strains of blood or protein in the urine. This is not evident to the naked eye and is usually found only through urine tests.

These symptoms will subside and go away completely as the disease clears. In some rare cases, where the impact is heavy on the kidneys, the patient might suffer from repetitive kidney-related issues. If identified in time and treated effectively, the impact on the kidney can be reduced.

Since HSP develops after a respiratory infection, if your child has recently recovered from a respiratory infection and has a new rash on the legs along with joint pains and a tummy ache, it can indicate HSP.

Causes of Henoch Schonlein Purpura in Children

The actual cause for Henoch Schonlein Purpura is still unknown. Doctors and researchers have not been able to identify any single organism as responsible for the onset of HSP in children or adults.

HSP is usually seen as an aftereffect of a mild respiratory illness. It can occur about 10 days after the illness. It is more prevalent during the spring season (winter and spring is when many respiratory infections occur).

HSP can sometimes be triggered by certain medications that were prescribed to treat other infections or diseases such as chickenpox, measles, strep throat, hepatitis. Some studies even associate HSP with certain vaccinations but nothing has been confirmed yet.

HSP occurs when the immune system starts attacking the body after a respiratory illness. So, it is classified as an autoimmune disorder. This immune response could be to respiratory illness or certain medications as mentioned earlier. Sometimes, it can be a response to insect bites too.

[Read : 5 Types Of Hepatitis In Children And Their Prevention]

Complications of Henoch Schonlein Purpura in Children

HSP resolves on its own in a few days. Many children recover fully without any medical intervention or special treatments. In some cases, analgesics or certain steroids are used to keep the inflammation under control. Sometimes a child might require just paracetamol to help relieve the pain.

1. Impact on Kidneys

In some rare cases, the disease can affect the kidney or other organs such as the brain or the spine. Impact on the digestive tract is a classical symptom, which causes abdominal pain and vomiting in affected children.

If the inflammation affects the kidneys and is not treated well in time, it can have a lasting impact on the kidneys. It can lead to various kidney problems or even complete kidney failure that might require a transplant. This is very rare though.

2. Impact on Bowels

In case of bowel obstruction, it can cause bloody stools or severe abdominal pain due to bleeding in the gastrointestinal tract. In very rare cases, the bowel or the intestines might fold in (intussusception) causing severe pain. If this does not resolve on medications, surgery might be required.

3. Impact on Nervous System

If the disease impacts the spinal cord or the nervous system, the patient might experience severe headaches, dizziness, irritability, or ataxia.

These complications are not common in most children. They are very rare and develop when there is a delay in diagnosis and treatment. So do not fear the worst and get worried unless your doctor confirms the diagnosis and tells you there is a complication.

Diagnosis of Henoch Schonlein Purpura

So, how does a doctor diagnose HSP? The symptoms are easy to identify and the diagnosis is not very difficult if the classic symptoms are present. It can be tricky to diagnose if only one symptom is present along with symptoms of other diseases.

For example – In some rare cases where the central nervous system is affected, the patient might experience severe headaches, seizures, or convulsions. Now, just these symptoms can be easily confused with other illnesses.

1. Rash

The presence of the rash (Purpura) or the abdominal pain along with vomiting, especially soon after a respiratory illness, makes for an easy diagnosis.

2. Protein Deposit

HSP causes a deposit of a certain protein called immunoglobulin A (IgA) on the affected organs. If there are issues with the kidney or other organs, the doctor might ask for a skin sample to test for this protein deposit (skin biopsy). In case of severe issues with the kidney, a kidney biopsy might be required to confirm the diagnosis and start relevant treatment.

3. Pain

If the abdominal pain is severe along with bloody stools, the doctor might recommend an ultrasound of the bowel to check for obstructions.

4. Urine Test

If there is an involvement of the kidney, a urine test can be suggested. The urine test will help find out if there are traces of blood or protein in the urine (this happens when the kidney is affected). Remember, kidneys can get affected anywhere upto 6 months from the start of the rash. So, periodic urine testing is recommended in all children with HSP for 6 months.

Usually, a doctor confirms HSP if the child has a rash along with one of the four classic symptoms:

• Abdominal pain
• Arthritis
• Involvement of the bowels
• Deposit of IgA on skin biopsy

Further tests are ordered to confirm the diagnosis and to understand the extent of the impact on the body.

[Read : Juvenile Idiopathic Arthritis in Children ]

Why is There a Delay in Diagnosis of HSP?

So, if it is so easy to diagnose and there is not much room for confusion when the classical symptoms are present, why is there a delay in some cases? Why does it lead to certain complications?

Well, different patients will consult the doctor at different stages of the disease. If you go at the right time, it might make the diagnosis a little easier. In some cases, the symptoms may not be very clear or might be accompanied by other symptoms. In such cases, the doctors will have to rule out other diseases or illnesses and finalize a diagnosis. They cannot rush to diagnose a common rash caused by some insect bite, to be HSP.

Patients may show other symptoms before the classic rash surfaces. For some, it may take more time and the various parts of the body might be under attack in the meanwhile. If a particular organ like the kidney is affected before the rashes appear, the doctor will have to treat both HSP as well as the kidney problem.

It is important that you convey everything to your doctor. The purple rash or the sudden bleeds might be very alarming. You may forget to inform the doctor about the respiratory infection your child experienced a few weeks ago, simply because they have recovered from it and this is something that looks scarier. Always remember, you need to give your doctor the history of your child’s health or illnesses, in case you are consulting a different doctor.

How to Prevent Henoch Schonlein Purpura in Children?

Since the actual cause of Henoch Schonlein Purpura is still unknown, there is no proven way to prevent this condition. Steroids do not prevent HSP and they cannot prevent the attack on the kidneys in children with HSP. The best thing is to get a timely diagnosis and initiate appropriate treatment based on the problems in a given child.

Treatment For HSP in Kids

In most cases, the disease goes away on its own, without any serious treatment or medications. Only paracetamol for pain relief, and simple skin creams may be sufficient in many children. In a few cases though, the doctor might ask admit your child into a pediatric facility to keep a close watch on them, especially if the abdominal pain is severe or the kidneys are affected. Since HSP can aggravate quickly, they will monitor the child constantly. Once the symptoms subside, they will discharge your child.

If your child is diagnosed with HSP, here is what you can expect in terms of treatment:

• Supportive and symptomatic care like
• Pain management
• Medical care for skin lesions or ulcers
• IV fluids to rehydrate
• Acetaminophen
• Non-steroidal anti-inflammatory drugs (only if there are no gastrointestinal or renal issues) to help alleviate joint pains and fever.

In order to manage the symptoms of HSP, the doctor might prescribe

1. Corticosteroids

This steroid help in reducing the severity or duration of abdominal pain in the initial weeks. Also help in treating kidney issues. When the doctor prescribes corticosteroids, the patient will need assistance to slowly taper down the dosage. They cannot stop it overnight.

[Read : Steroids in Children]

2. Immunosuppressants

Immunosuppressants are prescribed to prevent any irreversible glomerular fibrosis and kidney impairment, in case the kidneys are involved.

3. Regular Urine Analysis

Regular Urine Analysis is important to ensure there is no renal involvement. The urine tests can show if there is blood or protein in the blood. If there are even mild traces, the doctors will have to start treatment accordingly, to avoid further damage to the kidneys.

When to Seek Doctor’s Help?

If your child starts showing one of the four classic symptoms, it is time to consult a doctor. Since the Henoch Schonlein Purpura is more common among kids below the age of 10, it is more important to consult your pediatrician even if it is a mild rash.

Never try to self-diagnose or self-medicate. Though the signs and symptoms might be standard, there could be other underlying issues, which only a doctor can identify. Also, if you delay too much, some symptoms might subside or damage to organs in the body might progress very quickly. Both cases might complicate the diagnosis and make it difficult to treat the condition effectively.

HSP is a self-limiting disease, but it can reappear in some rare cases. If you notice the rashes or pain returning, do not discard them as after-effects of the diseases or the treatment. Check with your doctor immediately. Ensure you do not wait long until you see your doctor as the condition can progress quickly.

When the condition recurs, it is usually milder and not very alarming. In most cases, it is only the rashes that reappear, not the joint pains. The rashes will also clear out on their own. However, it is always safer to get an expert opinion. The doctor might just let it be or prescribe something mild to help with the rash and irritation.

You cannot expect a child to accept the recurrence of the condition like adults. They may not cooperate as well as they did the first time. A little extra help in the form of medications or a doctor’s advice can be very useful.

Recovery Time For HSP

The rashes and joint pains will eventually subside and clear away completely. For some, it may take just 4 weeks and for some, it can go on for about six weeks. In case of complications with the kidney, the treatment might extend further to ensure there is no permanent damage to the kidneys.

This is why it is important to follow up with regular urine analysis for a while. The rashes might subside but the damage could be extending on the inside. In very rare cases, the damage to the kidneys could be severe and might require further treatment or a transplant.

Once your child has fully recovered from HSP and has been given clearance by the doctor, you will have follow-up check-ups. The doctor will ask for regular check-ups with urine analysis (urine routine test) every fortnightly for the first 6 months. Further follow-ups will be needed in case, kidneys are affected.

Conclusion

Henoch Schonlein Purpura is not a very common disease you might come across as a parent. To see those purple spots or bleeds can be very alarming for a parent, especially if your child has just recovered from a respiratory illness.

It is important to not lose heart or show fear in front of your child. Children observe everything and absorb your emotions. If you are going to be scared and worried constantly, they will assume it is something very dangerous and might not cooperate with the treatment or get traumatized.

Also remember, your child is going to be very irritable and will have tantrums. Yes, even a 10-year-old can refuse to cooperate for hospitalization or treatment. It is important to maintain your calm and help them through this rough patch.