Henoch Schonlein Purpura (HSP)

What is Henoch Schonlein Purpura (HSP)

Henoch Schonlein purpura is a condition that causes swelling and inflammation of small blood vessels (vasculitis). This inflammation makes blood vessels leak, which causes a rash.

Henoch Schonlein Purpura is a systemic leukocytoclastic vasculitis affecting small vessels, characterized by IgA immune deposits.

The rash can appear anywhere, but it is most common on the arms, legs, and buttocks.

Henoch Schonlein Purpura can also cause:

• Bleeding into your bowel and kidneys.
• Joint inflammation.
• Kidney damage. It can lead to long-term or end-stage kidney disease and kidney failure.

How common is Henoch Schonlein Purpura?

The incidence increases in the fall and winter, possibly due to increased infectious or allergic triggers.

The classic presentation is a tetrad of skin rash, abdominal pain, arthralgias, and hematuria/proteinuria.

Henoch Schonlein Purpura has a peak incidence at ages 4 to 6 and shows a 2:1 male-to-female predilection.

It is the most common cause of vasculitis in children.

What are the causes?

An overactive disease-fighting system (immune system) causes HSP. This means that something triggers your immune system to produce proteins (antibodies) that attack your blood vessels. This is called an autoimmune reaction. Triggers for HSP may include:

• Infections from certain viruses or bacteria.
• Certain medicines.
• Certain types of cancer.

What increases the risk?

You are more likely to develop this condition if:

• You have allergies.
• You have a family history of HSP.
• You are male.

What are symptoms of Henoch Schonlein Purpura?

The main symptom of this condition is a skin rash that may look like raised bruises or dots. The rash changes color over time from reddish-purple to rust-colored. The rash color does not go away when you press the spots.

Other symptoms include:

• Fever.
• Tiredness (fatigue).
• Abdomen (abdominal) pain. This may include nausea, vomiting, and diarrhea.
• Blood or protein in your urine.
• Blood in your stool.
• Swollen and painful joints, especially the knees and ankles.
• Swollen testicles in men.

The four classic clinical features are as follows:

• Skin: Palpable purpura affecting mainly the forearms, lower limbs, and buttocks. Skin manifestations usually predominate in children.

• Gastrointestinal: Bowel vasculitis causing colicky abdominal pain. Gastrointestinal bleeding may occur.

• Joints: Symmetric polyarthralgia typically limited to the knees and ankles.

• Kidney: This may be clinically and pathologically indistinguishable from IgAN. Kidney involvement is more commonly seen and more severe in older children and adults than in young children.

How is Henoch Schonlein Purpura diagnosed?

This condition may be diagnosed based on:

• Your symptoms and medical history.
• A physical exam.
• Urine tests to check for blood or protein.
• Blood tests to check for complications and to make sure there is not another cause of your illness.
• Removing a small skin sample and examining it under a microscope (skin biopsy).
• Kidney biopsy.

How is Henoch Schonlein Purpura treated?

There is no cure for this condition, but treatment can help to relieve symptoms. Treatment may include:

• Staying in bed (bed rest).
• Raising (elevating) affected arms and legs above the level of the heart.
• Drinking more fluids.
• Taking medicines to reduce inflammation and pain. These may include:
  • NSAIDs to relieve aches and fever.
  • Corticosteroids to lessen inflammation and relieve pain.

If you have severe kidney damage, you may need medicines that reduce the activity of the immune system (immunosuppressants). Kidney failure may be treated with a process to clean the blood of wastes, salt, and extra fluid (dialysis).

Follow these instructions at home:

• Take over-the-counter and prescription medicines only as told by your health care provider.
• Follow instructions from your health care provider about activity restrictions or bed rest.
• Raise (elevate) affected arms and legs above the level of your heart when you experience symptoms.
• Drink enough fluid to keep your urine pale yellow.
• Keep all follow-up visits as told by your health care provider. This is important.

Contact a health care provider if:

• Your symptoms get worse, and medicines do not help.
• You have a little bit of blood in your urine or stool.

Get help right away if:

• You have severe abdominal pain.
• You vomit repeatedly.
• You have heavy bleeding in your urine or stool.
• You are not able to urinate.
• You have abdominal pain and are not having any bowel movements.
• You have a seizure.
• You have mental confusion.
• You have chest pain.
• You have trouble breathing or shortness of breath.

Summary

• Henoch–Schonlein purpura (HSP) is a condition that causes swelling and inflammation of small blood vessels (vasculitis).
• An overactive disease-fighting system (immune system) causes HSP.
• A skin rash is the most common symptom.
• There is no cure for HSP, but treatment may help to relieve symptoms.