Henoch-Schönlein purpura (HSP) is a disease that causes small blood vessels in the

skin to leak because of inflammation. The

primary symptom is a rash that looks like

many small raised bruises on the legs. The

rash is most often on the legs and buttocks,

but it can appear on other parts of the

body. Some people with HSP also develop

abdominal pain or joint pain (arthritis).

The kidneys may be affected as well,

causing blood or protein in the urine.

HSP can occur any time in life, but it

usually happens in children between the

ages of 2 and 11.

In most cases, HSP lasts 4 to 6 weeks, with

no long-term consequences. Sometimes

symptoms come and go during this time

period. About one in three people have

more than one episode (recurrence) of

HSP. Recurrences usually occur within a

few months and are usually less severe than

the initial episode. 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.

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 the problem.

What are the causes of HSP?

The causes of HSP are 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 your 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, 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.

National Kidney and Urologic Diseases Information Clearinghouse

Henoch-Schönlein

Purpura

National

Institute of

Diabetes and

Digestive 

and Kidney

Diseases

NATIONAL 

INSTITUTES

OF HEALTH

U.S. Department

of Health and

Human Services

The primary symptom of Henoch-Schönlein purpura is a

rash that looks like many small raised bruises on the legs.
What are the symptoms

of HSP?

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 you press on it.

• 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 percent 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

they’re present.

• Kidney involvement. Blood in the

urine (hematuria) occurs in about 40

percent 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.

How is HSP diagnosed?

When a typical rash, abdominal pain, and

arthritis are present, the doctor can easily

recognize HSP. But many people with HSP

have only the rash, which can sometimes be

seen as a symptom of other conditions and

may delay the clinical diagnosis of HSP.

The doctor may need to perform a series of

tests to confirm a diagnosis of HSP, as no

single test for HSP exists.

• Blood test. Elevated levels of blood

urea nitrogen and creatinine, which

are waste products that are normally in

the blood at low levels, indicate that

the kidneys are affected. Healthy

kidneys filter urea and creatinine

from blood.

• Urine sample. A urine sample is

needed to check for hematuria, which

is blood in the urine, and to check for

proteinuria, which is protein in the

urine. Blood and high levels of protein in the urine indicate damage to

the kidneys.

• Skin biopsy. If other testing is inconclusive, and a diagnosis is required,

the doctor may take a small sample of

your skin to examine with a microscope. 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 off infections.

• Kidney biopsy. 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 the doctor decide

what specific medicines, if any, need

to be given for the kidney disease.

Very few patients with HSP need a

kidney biopsy.

2 Henoch-Schönlein Purpura
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 the

doctor identify HSP.

How is HSP treated?

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, you can use overthe-counter medicines, such as acetaminophen (Tylenol), for the pain. In some

patients with severe arthritis, the doctor

may prescribe prednisone, a steroid medicine. As mentioned earlier, the rash and

joint symptoms usually go away after 4 to 6

weeks without causing permanent damage.

Severe problems with the bowels are rare in

HSP, especially in younger children. If you

have severe pain or severe bleeding in the

digestive tract your doctor may prescribe

prednisone, or the problem may need to be

corrected with surgery.

Your doctor will check your 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, your doctor will refer you 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.

What is the long-term

outlook?

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 usually occur within a

few months and are usually less severe than

the initial episode. When the symptoms

recur or last longer than 6 weeks, they can

be very frustrating and uncomfortable. The

long-term outlook is still good, however, as

long as your kidneys are healthy.

If you develop progressive kidney disease,

you will need to have regular checkups to

monitor your kidney function. In the early

stages of kidney disease, you may not have

any symptoms, but blood and urine tests

may show that your kidney function is

declining. If you continue to have blood

and protein in your urine, you are at greater

risk of developing chronic kidney disease.

Between 20 and 50 percent of children with

HSP develop some kidney problems, but

only 1 percent progress to total kidney failure. Progression to kidney failure may take

as long as 10 years.

Hope Through Research

Through its Division of Kidney, Urologic,

and Hematologic Diseases, the National

Institute of Diabetes and Digestive and

Kidney Diseases (NIDDK) supports several

programs and studies devoted to improving

treatment for patients with progressive kidney disease and kidney failure. The NIDDK maintains the Pediatric Nephrology

Program, which supports research into the

causes, treatment, and prevention of kidney

diseases in children, including congenital

malformations of the urinary tract, polycystic disease, primary glomerular disease,

and postinfectious glomerulonephritis.

3 Henoch-Schönlein Purpura

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Clearinghouse

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Phone: 1–877–22NIAMS (226–4267)

or 301–495–4484

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U.S. DEPARTMENT OF HEALTH 

AND HUMAN SERVICES

National Institutes of Health

NIH Publication No. 06–5860

September 2006

National Kidney and

Urologic Diseases

Information Clearinghouse

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Email: nkudic@info.niddk.nih.gov

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The National Kidney and Urologic Diseases Information Clearinghouse (NKUDIC) is a service of the

National Institute of Diabetes and Digestive and Kidney

Diseases (NIDDK). The NIDDK is part of the National

Institutes of Health under the U.S. Department of

Health and Human Services. Established in 1987, the

Clearinghouse provides information about diseases of

the kidneys and urologic system to people with kidney

and urologic disorders and to their families, health care

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works closely with professional and patient organizations and Government agencies to coordinate

resources about kidney and urologic diseases.

Publications produced by the Clearinghouse are carefully reviewed by both NIDDK scientists and outside

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Flynn, M.D., of the American Society of Pediatric

Nephrology (ASPN), for coordinating the review of this

publication by the ASPN’s Clinical Affairs Committee:

Barbara Fivush, M.D. (co-chair), Steve Wassner, M.D.

(co-chair), John Brandt, M.D., Deepa Chand, M.D., Ira

Davis, M.D., Stuart Goldstein, M.D., Ann Guillot, M.D.,

Deborah Kees-Folts, M.D., Juan Kupferman, M.D.,

Gary Lerner, M.D., Tej Mattoo, M.D., Alicia Neu, M.D.,

Cynthia Pan, M.D., William Primack, M.D., and Michael

Somers, M.D.

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