Henoch-Schonlein Purpura

What is Henoch-Schönlein purpura?

Henoch-Schönlein purpura (HSP) is a form of vasculitis, a condition that involves inflammation of the blood vessels. It can affect any organ of the body.

HSP is the most common form of vasculitis in childhood and affects about 20 in 100,000 children. It occurs most commonly in children ages 2-6, although it can occur at any age.

Patients with Henoch-Schönlein purpura develop a characteristic bruise-like rash on their arms and/or legs. In most cases, the condition does not require treatment; it will resolve on its own and the child will recover completely.

However, some children with Henoch-Schönlein purpura will develop arthritis and abdominal pain. These children can be treated with pain control medications.

The most serious potential complication of Henoch-Schönlein purpura is kidney damage — which can be irreversible.  For this reason, children with HSP should be carefully monitored for the first 6 months after diagnosis. Testing should include regular blood pressure checks and urine analysis.

About one third of children diagnosed with Henoch-Schönlein purpura will develop recurrent symptoms of Henoch-Schönlein purpura, although most recurrent episodes are less severe than the initial episode. However, recurrent episodes of Henoch-Schönlein purpura can last up to a year after the initial diagnosis.

Causes

The cause of Henoch-Schönlein purpura is unknown.

Environmental triggers, such as an infections, have been proposed as a trigger for initiating Henoch-Schönlein purpura, although such triggers are not always identified.

A genetic component to the disease has also been proposed, although no clear link has been made between an individual gene and the development of Henoch-Schönlein purpura.

More research is needed to understand the cause of this disease.

Signs and symptoms

Each child with Henoch-Schönlein purpura may experience symptoms differently. Common symptoms include:

  • Purpura: hemorrhage (bleeding) into the skin, mucous membranes and internal organs
  • Pain and inflammation of the joints
  • Abdominal pain
  • Gastrointestinal bleeding at any place along the GI tract — mouth, esophagus, stomach or intestines.
  • Inflammation of the kidneys (nephritis)
  • Swelling just below the skin (subcutaneous edema)
  • Dysfunction of the brain (encephalopathy)
  • Inflammation of the testicles (orchitis)
  • Bleeding from the lungs

The symptoms of Henoch-Schönlein purpura may resemble other conditions or medical problems. Always consult your child's doctor for a diagnosis.

Testing and diagnosis

Henoch-Schönlein purpura is usually diagnosed based on the presence of several symptoms. These include:

  • Palpable purpura: hemorrhage (bleeding) into the skin or mucous membranes and other tissues
  • Abdominal pain
  • Kidney disease
  • Arthritis

A platelet count should be obtained in cases of suspected HSP to ensure that it is normal. If the presentation is not typical, a biopsy of the involved area may be required.

In addition, ultrasound (a diagnostic imaging technique that uses high-frequency sound waves and a computer to create images of blood vessels, tissues and organs) may be used to examine the gastrointestinal tract for presence of the disease.

Treatment

Specific treatment for Henoch-Schönlein purpura will be determined by your child's doctor based on:

  • Your child's overall health and medical history
  • Extent of the condition
  • Your child's tolerance for specific medications, procedures and therapies
  • Expectation for the course of the disease
  • Specific organs that are affected
  • Your opinion or preference

Treatments for Henoch-Schönlein purpura includes supportive care, such as:

  • Adequate hydration, or fluid intake
  • Careful attention to nutrition
  • Pain control with medications such as acetaminophen
  • Glucocorticoids (to control inflammation)
  • Blood pressure medication if elevated blood pressure occurs

Follow-up care

Patients diagnosed with Henoch-Schönlein purpura will need to be followed closely by their doctor for the development of kidney involvement even after other symptoms of the disease have subsided.

In children with severe Henoch-Schönlein purpura or disease affecting the kidneys, they may need to see pediatric subspecialty doctors, such as a rheumatologist or nephrologist (kidney doctor).

Outlook

Most children with Henoch-Schönlein purpura will recover completely without long-term consequences. 

About a third of children with HSP will experience a recurrence of symptoms within the first year after disease onset. In most cases, the recurrence is less severe and lasts a shorter period of time than the initial onset of symptoms.

Women with a history of Henoch-Schönlein purpura, regardless of prior kidney involvement, are at increased risk to develop preeclampsia during pregnancy.

In rare cases, HSP can lead to permanent damage to the kidneys, which may require life-long management by a nephrologist.