Henoch Schonlein Purpura

What is Henoch Schonlein Purpura (HSP)?

Henoch Schonlein Purpura may also be called Immunoglobulin A vasculitis (IgA vasculitis). IgA is a type of protein made by white blood cells. Vasculitis means blood vessels become inflamed; this inflammation occurs because of IgA.

Purpura are purple spots seen on the skin when inflamed, damaged small blood vessels bleed just below the skin. These purple spots are often raised and look similar to bruises. These spots can also be found on the surface of the small intestine, stomach and colon. 

Who gets Henoch Schonlein Purpura?

Most cases occur in kids and teenagers. It is less likely to occur in summer months. Adults can be affected, but this is much less common.

What causes Henoch Schonlein Purpura?

The exact cause of HSP is not known, but HSP often occurs after an infection, including strep throat or viruses like the common cold or flu. Not all people who have a viral infection will develop HSP. Multiple factors including the immune system, the environment, and specific infection probably all contribute to development of HSP. 

What are the signs and symptoms of Henoch Schonlein Purpura?

Common symptoms include palpable purpura (raised, purple, bruise-like purple spots), muscle pain, joint pain, belly pain or kidney disease. In most patients, raised purple spots (purpura) are the first symptom, but not all. Purple spots are most commonly found on the legs and buttocks.

Patients may have swelling, especially around their eyes, feet, and legs. Joint pain is common and there may be swelling at the joint. Pain can move between different joints and may be so severe that affected children are unable to walk. 

When the blood vessels in the kidneys are affected, it can cause there to be blood in the urine; sometimes there may also be protein in the urine. The symptoms may not all occur at the same time and can appear over the course of days or weeks. 

What GI symptoms are associated with Henoch Schonlein Purpura?

About half of kids with HSP will report belly pain, nausea, or vomiting. They may have blood in their stool or develop intussusception. Intussusception occurs when the intestine “telescopes” or one portion slides into another and becomes stuck. This can cause abdominal pain.  The pain is often described as crampy and severe.

Rarely, patients can have severe belly pain from their pancreas becoming inflamed (pancreatitis) or a hole (ulcer) in their intestine developing.  Decreased appetite is common with either of these conditions. With pancreatitis, patients often have intense vomiting. Their pain is often located in the middle of their belly, just below where the ribs end. If patients have a hole in their intestine, they will have severe belly pain and may develop a fever. Some children may have low protein levels because their protein is being lost through the inflamed gastrointestinal tract.

How is Henoch Schonlein Purpura (HSP) Diagnosed?

The diagnosis is made clinically, which means your doctor makes the diagnosis based on symptoms and exam findings.  Lab tests often are not specific for HSP.

If there is confusion on the diagnosis based only on clinical symptoms, labs or biopsies may help your doctor. Labs will show normal ability of the blood to clot and normal platelet counts. Rarely a biopsy of the skin or kidney be obtained to look for immunoglobulin A attached to the blood vessels. 

How is Henoch Schonlein Purpura treated?

Henoch Schonlein Purpura usually resolves without any treatment needed.

For joint pain or abdominal pain, non-steroidal anti-inflammatory medicines like ibuprofen may be helpful. For severe pain, some patients may get oral steroids like prednisone. 

With severe belly pain, kidney damage, high blood pressure or for patients unable to drink enough fluids by mouth to stay hydrated, hospitalization may be necessary to provide IV fluids, medicines to help control pain or blood pressure and to closely monitor kidney function.

If patients have intussusception, they may require a special type of enema where air is injected into the rectum. This can cause the “telescoped” portions of the small intestine to come apart. In rare cases that cannot be fixed with an enema, surgery may be required. 

How long does HSP last?

Most patients return to normal after a month or less of symptoms. About one out of every three patients with HSP will have a second episode that is usually less severe than the first. Even after symptoms have gone away, patients’ kidney function should be carefully monitored by their pediatrician or kidney doctor by checking blood pressure and their urine. Some children may need blood tests to monitor their kidney function too. 

Authors: Arathi Lakhole, MD and Erik Andrewski, MD
Editor: Christine Waasdorp, MD
January 2024