Henoch-Schönlein Purpura

Purpura on the right lower leg of a child. Source: Wikimedia Commons

Henoch-Schönlein Purpura (HSP} is a condition characterized by an inflammation of the small blood vessels called capillaries. This inflammation, also known as vasculitis, causes a purple or red rash (purpura). The vasculitis may also affect the kidneys and bowel. HSP generally lasts 4 to 6 weeks, with no long-term consequences. Sometimes symptoms come and go during this time period and one in three people have recurrent (more than one episode) HSP. The syndrome is usually seen in children, but it may affect people of any age and is more common in boys than in girls.

Other names

• Anaphylactic purpura

Signs and Symptoms

HSP has four main symptoms:

Rashes and bruising

Leaking blood vessels in the skin cause rashes that look like bruises or small red dots to develop on the legs, buttocks, and back of the arms. The rash may first look like hives, then change to look like bruises. Rarely, the rash may spread to the upper part of the body, but it is usually on the parts of the body that hang down, like the legs, buttocks, elbows, and even earlobes. The rash does not disappear or turn pale when pressure is applied to the skin.

Abdominal pain

About two-thirds of people with HSP experience pain in the stomach that may cause vomiting or blood in the stool. This pain and bleeding can vary from mild to severe.

Arthritis

About 80% of people with HSP have pain and swelling in their joints, usually in the knees and ankles, less frequently in the elbows and wrists. These joint symptoms have no long-lasting effects, although they can be very uncomfortable while present.

Kidney involvement

Blood in the urine (hematuria) occurs in about 40% of people with HSP. Often the blood cannot be seen by the naked eye, but it can be measured with a laboratory test called a urinalysis. In most people the hematuria goes away without permanent kidney damage. Protein in the urine or development of high blood pressure (hypertension) suggests more severe kidney problems.

Causes

The causes of HSP is not fully understood. One theory is that it may develop as an immune response to an infection. In other words, the body's infection-fighting system, the immune system, continues to attack cells after the infecting organisms are gone. For example, HSP may develop after a cold. The cold germs cause the immune system to take action. Once the immune cells have rid the body of the germ cells, they normally rest. But with HSP, the immune cells continue to attack other cells in the body. This theory is also based on the fact that, in many cases, HSP symptoms recur or worsen during upper respiratory infections.

HSP has also been associated with insect bites and exposure to cold weather. Other cases [have developed after a person received vaccination for ]typhoid fever, measles, cholera, hepatitis B, or yellow fever. Some foods, drugs, or other chemical toxins may trigger HSP as well. Often no cause can be found.

Diagnosis

When a typical rash, abdominal pain, and arthritis are present, HSP can be more easily diagnosed. Many people with HSP only present with a rash, which can sometimes be seen as a symptom of other conditions and may delay the clinical diagnosis of HSP.

No single test exists for the diagnosis of HSP. Common tests include:

• Blood tests which look for elevated levels of blood urea nitrogen and creatinine, waste products that are normally present in the blood at low levels. Abnormally high levels of these chemicals indicate that the kidneys are affected by HSP as healthy kidneys filter urea and creatinine from the blood.
• A urine sample to check for hematuria (blood in the urine) and proteinuria (protein in the urine). Blood and high levels of protein in the urine indicate damage to the kidneys.
• If other testing is inconclusive, and a diagnosis is required, the doctor may take a small sample of the skin to examine with a microscope. This test is known as a skin biopsy. The biopsy may reveal large numbers of white blood cells in the skin and deposits of IgA, one of the proteins normally made by the immune system to help fight infection.
• When the kidneys are affected by HSP, the nephrologist (kidney specialist) may take a small sample of kidney tissue to examine with a microscope. Examining the sample can help to make a decision about which specific medicines, if any, need to be given for the kidney disease. Very few patients with HSP need a kidney biopsy.

Several diseases share some of the symptoms of HSP. But consistent physical exam findings, along with blood, urine, and skin test results taken together, can help to identify HSP.

Treatment

There is no specific treatment for HSP. The main goals of treatment are to relieve symptoms such as joint pain, abdominal pain, or swelling. In most cases, over-the-counter medicines such as acetaminophen can be taken for the pain. In some people with severe arthritis, the doctor may prescribe prednisone, a steroid medicine. As mentioned earlier, the rash and joint symptoms usually go away after four to six weeks without causing permanent damage.

Severe problems with the bowels are rare in HSP, especially in younger children. If there is severe pain or severe bleeding in the digestive tract the doctor may prescribe prednisone, or the problem may need to be corrected with surgery.

The doctor will check kidney function with blood and urine tests even after the main symptoms of HSP disappear. People who develop kidney disease usually show signs within 3 to 6 months after the initial rash appears. If signs of kidney disease appear, people are usually referred to a nephrologist, who may prescribe drugs to suppress the immune system. These immunosuppressive drugs may keep kidney disease from progressing to permanent kidney failure.

Living with Henoch-Schönlein Purpura

• Patients may be given a short course of prednisone (a steroid) or an NSAID (a non-steroidal anti-inflammatory medication such as naproxen or ibuprofen). These medicines are given only if the kidneys are functioning normally.
• Elevating the legs may be helpful to keep the purpura from developing.
• Patients should be careful not to expose themselves to any type of injury. Even the most minor activies such as jogging can cause cause a worsening of symptoms.

Related Problems

Some patients can go from having blood (hematuria) in the urine to a decrease in kidney function called renal insufficiency. HSP patients who experience hematuria may be followed more closely, with regular testing of the urine for blood and protein.

Another rare complication of HSP is intussusception of the bowel, or intestine. With this condition, a section of the bowel folds into itself like a telescope. The bowel may become blocked as a result. Surgery may be needed to correct this problem.

Clinical Trials

No ongoing studies related to Henoch-Schönlein purpura were found at this time.

Research

Current research

• The efficacy of combining corticosteroid medication in combination with cyclophosphamide versus corticosteroid alone in the treatment of severe Henoch-Schönlein purpura. ^[1]
• A study is underway to compare compare pulsed doses of methylprednilosone and cyclosporine A for their efficacy in the treatment of HSP nephritis. ^[2]

Recent discoveries

• Henoch-Schönlein purpura can be diagnosed on endoscopy. This procedure can be a helpful tool for diagnosis in cases where gastrointestinal symptoms of HSP precede a rash. ^[3]
• A recent study from Japan found that oral and ear, nose, and throat diseases were commonly associated with HSP. Furthermore, the study found that early and agressive treatment of co-existing diseases lessened the complications of HSP. ^[4]
• The suspicion that meningococcal vaccine may be responsible for causing Henoch-Schönlein purpura was disproven in a recent study from New Zealand. ^[5]
• The gastrointestinal manifestations (diffuse mucosal edema, erythema, petechia or multiple irregular ulcers, especially in the second portion of the duodenum or in the terminal ileum) are discussed after a study reviewing pathology and endoscopy reports. ^[6]
• A case of HSP during pregnancy is reviewed. This is only the seventeenth reported case of HSP in pregnancy. ^[7]

Expected Outcome

Most cases of HSP resolve within 4 to 6 weeks without long-term problems. About one in three people has a recurrence of HSP. Recurrences, found in 33% of patients, usually develop within the first few months after resolution of the first episode of HSP. Recurrences usually are usually less severe than the initial episode of HSP.

If progressive kidney disease develops, it will be necessary to have regular checkups to monitor kidney function. In the early stages of kidney disease, there may not be any symptoms, but blood and urine tests may show that kidney function is declining. If blood and protein are found in the urine, there is a greater risk of developing chronic kidney disease.

Between 20 and 50 percent of children with HSP develop some kidney problems, but only 1% progress to total kidney failure. Progression to kidney failure may take as long as 10 years.

Even when it lasts longer than a few months, HSP can still resolve completely. In a few cases, however, it can lead to kidney damage and permanent kidney failure. A person with severe kidney failure must receive a bloodcleansing treatment called dialysis or a kidney transplant if the damage is permanent.

History

Henoch-Schönlein purpura was named for Drs. Eduard Heinrich Henoch, a German pediatrician and Johann Lukas Schönlein, who was Dr. Henoch's professor of medicine. The men described the characteristic group of symptoms in the 1860s. The condition was previously known as Heberden-Willan disease. Reportedly, Dr. William Osler was the first to attribute HSP to an underlying allergic cause.

References

1. ↑ ClincicalTrials.gov. Efficacy of Steroid Versus Steroid Plus Cyclophosphamide for Severe Henoch-Schoenlein Purpura
2. ↑ ClinicalTrials.gov. HSP-Glomerulonephritis Trial: MP vs CyA
3. ↑ Nishiyama R, Nakajima N, Ogihara A. Endoscope Images of Schönlein-Henoch Purpura. Digestion. 2008 Aug 7;77(3-4):236-241. (Epub ahead of print) Abstract
4. ↑ Inoue CN, Nagasaka T, Matsutani S, Ishidoya M, Homma R, Chiba Y. Efficacy of early dental and ENT therapy in preventing nephropathy in pediatric Henoch-Schönlein purpura. Clin Rheumatol. 2008 Jul 24. (Epub ahead of print) Abstract
5. ↑ Sexton K, McNicholas A, Galloway Y. Henoch-Schonlein Purpura and meningococcal B vaccination. Arch Dis Child. 2008 Jul 23. (Epub ahead of print) Abstract
6. ↑ Zhang Y, Huang X. Gastrointestinal involvement in Henoch-Schonlein purpura. Scand J Gastroenterol. 2008 May 8:1-6. (Epub ahead of print) Abstract
7. ↑ Kalmantis K, Daskalakis G, Iavazzo C, Vranos A, Mesogitis S, Ansakalis A. J Obstet Gynaecol. 2008 May;28(4):403-5. Abstract

External Links

Pediatric Rheumatology European Society

American College of Rheumatology

American Society of Pediatric Nephrology