Henoch Schönlein Purpura (HSP)
Henoch Schönlein Purpura (HSP) is a type of vasculitis, which means inflammation of the blood vessels. The exact cause of HSP is unknown and there is no specific test to diagnose it. In 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 a rash. Bleeding into the stool and urine can also occur. In North America, HSP is the most common form of vasculitis in children. It is most common in ages 3 to 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 Are the Signs/Symptoms?
The classic symptom of HSP is a red to dark purple rash, called “purpura”, which is most severe on the legs and buttocks. Other symptoms include painful swelling around the joints, swelling under the skin and abdominal pain. Many children with HSP recover completely, but up to one-third of patients can develop kidney disease.
What Are Common Treatments?
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. Steroids given by mouth or through an IV infusion are typically given for severe abdominal pain. Many children with HSP can be treated by their primary care provider. 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. 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 Henoch Schönlein Purpura
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. Urine screening and monitoring of the blood pressure is recommended for at least 6 months after the initial diagnosis, so it is important to follow the screening instructions provided by your doctor.
Updated February 2023 by Cheryl Crow, MOT, OTR/L, and reviewed by the American College of Rheumatology.
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.