1. HENOCH-SCHONLEIN PURPURA (HSP)
Henoch-Schonlein purpura (HSP) is a disease involving inflammation of small blood
vessels. It most commonly occurs in children. The inflammation causes blood vessels in
the skin, intestines, kidneys, and joints to start leaking. The main symptom is a rash with
numerous small bruises, which have a raised appearance, over the legs or buttocks.
Although HSP can affect people at any age, most cases occur in children between the
ages of 2 and 11. It is more common in boys than girls. Adults with HSP are more likely
to have more severe disease compared to children.
HSP usually ends after four to six weeks -- sometimes with recurrence of symptoms
over this period, but with no long-term consequences. If organs such as the kidneys and
intestines are affected, treatment is often needed and it is important to have regular
follow-up to prevent serious complications.
Causes and Risk Factors for Henoch-Schonlein Purpura
The exact cause of HSP is not known. The body's immune system is believed to play a
role in targeting the blood vessels involved. An abnormal immune response to an
infection may be a factor in many cases. Approximately two-thirds of the cases of HSP
occur days after symptoms of an upper respiratory tract infection develop.
Some cases of HSP have been linked to vaccinations for typhoid, cholera, yellow fever,
measles, or hepatitis B; foods, drugs, chemicals, and insect bites. Some experts also
say that HSP is associated with the colder weather of fall and winter.
Symptoms of Henoch-Schonlein Purpura
The classic symptoms of HSP are rash, joint pain and swelling, abdominal pain, and/or
related kidney disease, including blood in urine. Before these symptoms begin, patients
may have two to three weeks of fever, headache, and muscular aches and pains.
Rarely, other organs, such as the brain, heart, or lungs, may be affected.
2. Here are some key details about the symptoms of HSP:
Rash. The rash usually appears in all patients with HSP. The initial appearance may
resemble hives, with small red spots or bumps on the lower legs, buttocks, knees, and
elbows. But these change to appear more like bruises. The rash usually affects both
sides of the body equally and does not turn pale on pressing.
Arthritis. Joint inflammation, involving pain and swelling, occurs in approximately three-quarters
of cases, particularly affecting the knees and ankles. It usually lasts only a few
days and does not cause any long-term, chronic joint problems.
Abdominal pain. In more than half of people with HSP, inflammation of the
gastrointestinal tract may cause pain or cramping; it may also lead to loss of appetite,
vomiting, diarrhea, and occasionally blood in the stool.
In some cases, patients may have abdominal pain before the rash appears. In rare
cases, an abnormal folding of the bowel (intussusception) may cause a bowel blockage,
which may require surgery to fix.
Kidney impairment. HSP can cause kidney problems, indicated by such signs as
protein or blood in the urine. This is usually only discovered on urine testing, since it
does not generally cause any discomfort.
In most patients, the kidney impairment is mild and goes away without any long-term
damage. It's important to monitor the kidney problems closely and make sure they clear
up, since about 5% of patients may develop progressive kidney disease. About 1% may
go on to develop total kidney failure.
Henoch-Schonlein Purpura Diagnosis and Treatment
The diagnosis of HSP may be clear when the typical rash, arthritis, and abdominal pain
are present. A doctor may order some tests to rule out other diagnoses, confirm the
diagnosis, and assess its severity.
Occasionally, when the diagnosis is uncertain, particularly if the only symptom is the
classic rash, your doctor may perform biopsies of the skin or kidney. Urine and blood
tests will likely be done to detect signs of kidney involvement and may need to be
repeated during follow-up to monitor any changes in kidney function.
Although there is no specific treatment for HSP, you can use over-the-counter pain
medicines, such as acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs),
3. such as ibuprofen and naproxen for joint pain. In some cases, corticosteroid medication
may be used.
The rash and joint pain will usually go away after four to six weeks without causing any
permanent damage. Bouts of the rash may recur in approximately one-third of cases,
but they are usually milder, do not involve joint and abdominal symptoms, and they
clear up on their own.
Henoch-Schonlein purpura (HSP) facts
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.
What is Henoch-Schonlein purpura (HSP)?
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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 a
purplish 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 season 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, including adults.
Medically Reviewed by a Doctor on 10/3/2012
