Henoch-Schönlein Purpura

What is Henoch-Schönlein purpura (HSP)?

Henoch-Schönlein purpura (say "hen-awk shern-line purr-purr-ah") is a disease that causes

the blood vessels in the skin to become inflamed (irritated and swollen). This inflammation is
called vasculitis. When the blood vessels in the skin are inflamed, they can bleed, causing a
rash that is called purpura. HSP also can affect blood vessels in the bowel and the kidneys.
When this happens, the intestines and the kidneys may also bleed.

What causes HSP?

The exact cause of HSP is unknown. Doctors think HSP happens when a person's immune
system doesn't fight an infection like it's supposed to. It might be triggered by bacterial or viral
infections, medicines, insect bites, vaccinations or exposure to chemicals or cold weather.
You may catch an infection that caused someone's immune system to respond with HSP, but
HSP itself isn't contagious. Doctors don't know how to prevent HSP yet.

HSP is more common in boys than in girls. It most commonly affects children 2 years to 11
years of age, but it may affect people of any age.

What are the symptoms of HSP?

HSP causes a skin rash that looks like small bruises or small reddish-purple spots on the
buttocks, around the elbows and on the legs. HSP can also cause pain in the joints (such as
knees and ankles), stomach pain, nausea, vomiting and diarrhea. The symptoms usually last
for about 4 to 6 weeks. If the blood vessels in the bowel and the kidneys get inflamed, HSP
can make you bleed when you have a bowel movement or when you urinate. Serious kidney
problems don't happen very often, but they can occur. If you or your child has symptoms of
HSP, see your doctor. In rare cases, an abnormal folding of the bowel called intussusception
(say "in-tuh-suh-sep-shun") can occur. This makes a blockage in the intestines that may need
surgery.

How is HSP treated?

There is no specific treatment for HSP. Medicines can help you feel better and treat an
infection that may have triggered HSP. Fortunately, HSP usually gets better without any
treatment. Anti-inflammatory medicines, such as ibuprofen (some brand names: Advil, Motrin)
or naproxen (brand name: Aleve), can help the pain in your joints. Sometimes medicines like
prednisone can help people who have severe joint and stomach pain.

Is HSP a serious disease?

Usually, HSP doesn't cause lasting problems and gets better on its own. About half of the
people who have had HSP once will get it again. A few people will have kidney damage
because of HSP. Your doctor may want to check urine samples several times after HSP goes
away to check for kidney problems. Be sure to see your doctor regularly during this time.
HSP
HENOCH-SCHONLEIN PURPURA
Overview
Henoch-Schonlein purpura (HSP) is named after two physicians who
independently discovered different aspects of this syndrome. HSP is
a vasculitis, an inflammatory disorder of the blood vessels.

The cause of HSP is unknown. It may be a post-infectious

phenomenon, as it often occurs after an upper respiratory illness; or
it may be some sort of hypersensitivity reaction, like allergic hives.
The disease occurs more frequently in children aged 2 to 8 years
old, and affects boys twice as frequently as girls.

Characteristics
The classic finding of HSP is a distinctive rash located on the back
surface of the legs. When it first starts, the rash may be variable in
appearance: it may consist of wheals or small red spots. The lesions
initially blanch under pressure but later lose this characteristic. The
rash eventually evolves into “palpable purpura.” This is the classic
stage of the rash: raised, purple bruises that do not blanch under
pressure.

The vasculitis of HSP affects blood vessels in all the organs of the
body. Swelling can occur anywhere, but commonly affects the
hands, ankles, and face. Arthritis of the large joints can be
prominent. Gastrointestinal symptoms such as abdominal pain or
bloody stools may occur. The kidneys may also be involved, causing
urinary symptoms and sometimes hypertension. Fever is present in
about half of all cases.

Recommendations
Give acetaminophen or ibuprofen as needed for pain and fever.
Acetaminophen does not have any anti-inflammatory properties.

Gastrointestinal, renal, or central nervous system involvement may

be treated with steroids.

Outcome
Although there is no specific treatment, the ultimate outcome is
excellent for most children.

The initial symptoms of the disease can last anywhere from a few
days to 6 weeks. Exacerbations and recurrences may occur.

In those who have renal involvement, about 25% can be expected to

have chronic symptoms.

Reasons for Concern

Seizures.
Confusion or disorientation.
Difficulty breathing.
Persistent abdominal pain.
Dehydration.
Persistent vomiting.
Vomit is green.
Blood in vomit.
Blood in stool.
Blood in urine.
Rash involves any mucus membranes: eyes, lips, mouth, inside of
nostrils, tip of penis, vaginal area.
Your child appears sick.
 What is Henoch-Schonlein purpura (HSP)?

Henoch-Schonlein purpura (HSP) is a form of blood vessel inflammation or

vasculitis. There are many different conditions that feature vasculitis. Each of the
forms of vasculitis tends to involve certain characteristic blood vessels. HSP affects
the small vessels called capillaries in the skin and frequently the kidneys. HSP results
in skin rash (most prominent over the buttocks and behind the lower extremities)
associated with joint inflammation (arthritis) and sometimes cramping pain in the
abdomen. Henoch-Schonlein purpura is also referred to as anaphylactoid purpura.

What causes HSP?

HSP occurs most often in the spring and frequently follows an infection of the throat
or breathing passages. HSP seems to represent an unusual reaction of the body's
immune system that is in response to this infection (either bacteria or virus). Aside
from infection, drugs can also trigger the condition. HSP occurs most commonly in
children, but people of all age groups can be affected.

What are symptoms of HSP?

Classically, HSP causes skin rash, pain in the abdomen, and joint inflammation
(arthritis). Not all features need be present for the diagnosis. The rash of skin lesions
appears in gravity-dependent areas, such as the legs. The joints most frequently
affected with pain and swelling are the ankles and the knees. Patients with HSP can
develop fever. Inflammation of the blood vessels in the kidneys can cause blood
and/or protein in the urine. Serious kidney complications are infrequent but can occur.

Symptoms usually last approximately a month. Recurrences are not frequent but do
occur.

How is HSP diagnosed?

HSP is usually diagnosed based on the typical skin, joint, and kidney findings. Throat
culture, urinalysis, and blood tests for inflammation and kidney function are used to
suggest the diagnosis. A biopsy of the skin, and less commonly kidneys, can be used
to demonstrate vasculitis. Special staining techniques (direct immunofluorescence) of
the biopsy specimen can be used to document antibody deposits of IgA in the blood
vessels of involved tissue.

What is the treatment for HSP?

While HSP is generally a mild illness that resolves spontaneously, it can cause serious
problems in the kidneys and bowels. The rash can be very prominent, especially on
the lower extremities.
 The treatment of HSP is directed toward the most significant area of involvement.
Joint pain can be relieved by antiinflammatory medications such as aspirin or
ibuprofen (Motrin). Some patients can require cortisone medications, such as
prednisone or prednisolone, especially those with significant abdominal pain or
kidney disease. With more severe kidney disease, involvement called
glomerulonephritis or nephritis, cyclophosphamide (Cytoxan), azathioprine (Imuran),
or mycophenolate mofetil (Cellcept) have been used to suppress the immune system.
Infection, if present, can require antibiotics.

What is the prognosis for patients with HSP?

The prognosis for patients with HSP is generally excellent. Nearly all patients have no
long-term problems. The kidney is the most serious organ involved when it is
affected. Rarely, patients can have serious long-term kidney damage or an abnormal
bowel folding called intussusception. Some patients have recurrences of symptoms,
particularly skin rash, for months to a year after the onset of the illness.

Recent data show that HSP in adults is generally more severe than in children. Adults
have more severe kidney involvement and can require more aggressive treatment. The
ultimate outcome, however, is usually very good for both adults and children.

Henoch-Schonlein Purpura At A Glance

• Henoch-Schonlein purpura is a particular form of blood vessel inflammation called vasculitis.

• Henoch-Schonlein purpura frequently follows an infection of the throat or breathing passages,
but it can be induced by certain medications.
• Henoch-Schonlein purpura causes skin rash, pain in the abdomen, and joint inflammation
(arthritis).
• The treatment of Henoch-Schonlein purpura is directed toward the most significant area of
involvement.
• The prognosis for patients with Henoch-Schonlein purpura is generally excellent.

References:

Primer on the Rheumatic Diseases. Springer, edited by John H. Klippel, et al., 2008.

Kelley's Textbook of Rheumatology. W B Saunders Co, edited by Shaun Ruddy, et al., 2000.

Last Editorial Review: 3/4/2009

Henoch-Schönlein Purpura (HSP)
What is Henoch-Schönlein purpura (HSP)?

Henoch-Schönlein purpura (HSP) is a form of vasculitis, a condition which involves inflammation of the
blood vessels. It is one of the most common forms of vasculitis in childhood. HSP is seen most
frequently in children between the ages of two and 11 years, and occurs more frequently in boys. A
family connection has been noted with HSP, where the disease has happened to two or more siblings of
the same family, either at the same time, or one after another.

What causes Henoch-Schönlein purpura?

As with the other forms of vasculitis, the cause of HSP is not known. HSP may be associated with an
upper respiratory tract infection or possibly an allergic reaction. Most children with HSP recover
completely.

What are the symptoms of Henoch-Schönlein purpura?

The following are the most common symptoms of Henoch-Schönlein purpura. However, each child may
experience symptoms differently. Symptoms may include:

• purpura - hemorrhage (bleeding) into the skin, mucous membranes, internal organs, and other
tissues.

• arthralgia - pain in the joints.

• inflammation of the joints.

• abdominal pain

• gastrointestinal bleeding - bleeding in the gastrointestinal, or GI, tract, which includes the
mouth, esophagus, stomach, and intestines.

• nephritis - inflammation of the kidneys.

• subcutaneous edema - swelling just below the skin.

• encephalopathy - dysfunction of the brain.

• orchitis - inflammation of the testicle(s).

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

How is Henoch-Schönlein purpura diagnosed?

Henoch-Schönlein purpura is diagnosed based on criteria established by the American College of

Rheumatology. These criteria include:

• palpable purpura - hemorrhage (bleeding) into the skin or mucous membranes and other
tissues.

• at onset of the disease, the patient is younger than 20 years of age.

• bowel angina, or pain in the abdomen which is worse after meals, or bowel ischemia
(decreased blood flow to an area), which may result in bloody diarrhea, among other signs.
 • the presence of certain cells when a tissue sample from the purpura is examined under the
microscope.

In addition, ultrasound (a diagnostic imaging technique which 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 for Henoch-Schönlein purpura:

Specific treatment for Henoch-Schönlein purpura will be determined by your child's physician 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 HSP may include:

• adequate hydration, or fluid intake

• careful attention to nutrition
• pain control with medications such as acetaminophen
• glucocorticoids (to control inflammation)