Overview

Henoch-Schonlein purpura, also known as IgA vasculitis, is a condition characterized by inflammation in the small blood vessels of the skin, gastrointestinal tract, and kidneys. Its symptoms often include a distinct purplish rash primarily found on the lower legs and buttocks, as well as joint pain. In some cases, it can lead to abdominal discomfort and inflammation in the joints. Although rare, severe kidney damage can occur as a complication of this condition.

While Henoch-Schonlein purpura can affect individuals of any age, it is most commonly observed in children under the age of 10. Fortunately, the condition typically improves without intervention. However, if the kidneys are affected, medical attention is necessary.

Symptoms

Henoch-Schonlein purpura has four primary characteristics, which are as follows:

  • Rash or purpura. The legs, feet, and buttocks acquire reddish-purple patches that resemble bruises. In regions of pressure, such as the sock line and waistline, the rash may be severe. It can also show up on the arms, face, and trunk.
  • Arthritis or painful and swollen joints. Henoch-Schonlein purpura patients frequently experience pain and swelling around the joints, particularly in the knees and ankles. Sometimes the characteristic rash appears one or two weeks before joint pain. When the disease clears, these symptoms go away and do not cause any long-term harm.
  • Digestive tract symptoms. In many cases, children with Henoch-Schonlein purpura may experience symptoms such as abdominal pain, nausea, vomiting, and bloody stools. It is important to note that these symptoms can occur prior to the appearance of the characteristic rash associated with the condition.
  • Kidney problems. The kidneys may also be impacted by Henoch-Schonlein purpura. The majority of the time, this manifests as protein or blood in the urine, which you might not even be aware of until a urine test is performed. Typically, the symptoms of Henoch-Schonlein purpura resolve as the illness subsides. However, in some cases, individuals may develop persistent kidney disease as a complication of the condition. It is important to monitor and manage the kidney function in such cases to prevent further complications.
  • Other symptoms: Fever is a possible symptom that can develop in patients with IgA vasculitis (Henoch-Schonlein purpura). Additionally, boys with this condition may experience painful swelling of the scrotum.

If you or your child experiences Henoch-Schonlein purpura with severe digestive tract complications, it is important to seek medical attention from your doctor. Additionally, if your child develops the characteristic rash associated with this condition, it is advisable to see a doctor promptly.

Causes

Henoch-Schonlein purpura (HSP) is a condition where small blood vessels in the body become inflamed, leading to bleeding in the skin, abdomen, and kidneys. The exact cause of this initial inflammation is unclear but may involve an inappropriate immune system response to various triggers. About half of HSP cases are preceded by upper respiratory infections like the common cold, while other triggers include chickenpox, strep throat, measles, hepatitis, certain medications, food, insect bites, and exposure to cold weather. Similarly, the cause of IgA vasculitis (IgAV), another term for HSP, is currently unknown. It often occurs after infections of the throat or respiratory system, primarily strep throat, and may involve an unusual immune system reaction to the infection, whether caused by bacteria or a virus.

Risk factors

Henoch-Schonlein purpura is more likely to occur in the presence of the following risk factors:

  • Gender. Males experience Henoch-Schonlein purpura somewhat more frequently than females do.
  • Age. Children under 10 are primarily affected by the illness.
  • Race. Black children are less prone than white and Asian children to have Henoch-Schonlein purpura.

Diagnosis

If the characteristic rash, joint pain, and digestive tract symptoms are present, your doctor can make a diagnosis of Henoch-Schonlein purpura. However, if one of these signs or symptoms is absent, your doctor may recommend conducting one or more of the following tests.

Lab tests

A diagnosis of Henoch-Schonlein purpura cannot be made by a single laboratory test, however specific tests can help rule out other illnesses and increase the likelihood of a Henoch-Schonlein diagnosis. They may consist of:

  • Blood examination. If your diagnosis is unclear based on your signs and symptoms, your blood may be checked.
  • Urine analysis. Urine tests can be conducted to detect signs of blood, protein, or other irregularities, indicating the functioning status of your kidneys.

Biopsies

Henoch-Schonlein purpura frequently leads to the accumulation of a specific protein called immunoglobulin A (IgA) in the affected organ. To examine this protein and make an accurate diagnosis, your doctor may extract a small section of your skin for laboratory testing. If the condition severely affects your kidneys, a kidney biopsy might be recommended by your doctor to assist in determining the most suitable treatment options.

Imaging tests

To rule out alternative causes of abdominal pain and to look for potential consequences like a bowel obstruction, your doctor could advise an ultrasound.

Treatment

Henoch-Schonlein purpura typically resolves spontaneously within a month without causing any long-term complications. Managing the symptoms can be achieved through adequate rest, staying hydrated, and using over-the-counter pain relievers as needed.

Medications

In some cases, corticosteroids like prednisone may be prescribed to alleviate joint and abdominal pain associated with Henoch- Schonlein purpura. It is important to note that these medications carry potential side effects, so it is crucial to have a discussion with your doctor regarding the risks and benefits before considering their use.

Surgery

If there is a complication such as bowel obstruction or rupture, surgical intervention may be necessary to address the issue.

Doctors who treat this condition