Henoch-Schoenlein Purpura (HSP) is a condition in which small
blood vessels get inflamed. This inflammation is called vasculitis
and usually affects the small blood vessels in the skin, bowel
and kidneys. The inflamed blood vessels may bleed into the skin
causing a deeply red or purple rash called purpura. They can also
bleed in the intestine or kidneys causing blood-stained stools
or urine (hematuria).
HSP was named after Drs Henoch and Schoenlein who both described
it independently more than one hundred years ago.
common is it?
HSP, although not a frequent illness of childhood, is the most
common systemic vasculitis in children aged between five and 15
years. It is more common in boys than in girls with a ratio of
There is no ethnic or geographic distribution for this disease.
Most cases in Europe and the Northern Hemisphere occur in winter,
but some cases are seen during fall or spring.
are the causes of the disease?
No one knows what causes HSP.
Infectious agents, such as viruses and bacteria, are thought to
be potential triggering factors for the disease, because it often
appears following an infection. However, HSP has also been seen
following prescription of medicines, insect bites, exposure to
cold, chemical toxins and the intake of specific foods that can
cause allergies. For these reasons the term allergic purpura was
previously used, as HSP was thought to be an allergic reaction
to all these agents. In some countries, it is also called rheumatoid
purpura due to symptoms related to joints and muscles (see the
section on symptoms below).
The presence of specific products of the immune system, such as
Immunoglobulin A (IgA), in lesions of HSP, suggest that an abnormal
response of the immune system attacks small blood vessels in the
skin, joints, the gastrointestinal tract, kidneys, central nervous
system or testis and causes the disease.
it inherited? Is it contagious? Can it be prevented?
HSP is not an inherited disease. It is not contagious and cannot
are the main symptoms?
The main symptom is a characteristic skin rash present in all
patients with HSP. The rash usually begins with small hives, red
patches or red bumps, which change to a purple bruise over time.
It is called palpable purpura, because the raised skin lesions
can be felt. Purpura usually covers the lower extremities and
buttocks, although some lesions may appear elsewhere in the body
(upper limbs, trunk etc).
Painful joints (arthralgia) or painful and swollen joints with
limitation of movement (arthritis), are found in the majority
of patients (>65%). Arthralgia and arthritis are accompanied
by soft tissue swelling and tenderness located near and around
the joints. Soft tissue swelling in hands and feet, forehead and
scrotum may occur early in the disease, particularly in very young
The symptoms effecting joints are temporary and disappear within
a few days.
When the vessels of the bowel become inflamed, abdominal pain
is present in more than 60% of cases. The pain appears intermittent
around the umbilical cord and may be accompanied by mild or severe
gastrointestinal bleeding (haemorrhage). Very rarely an abnormal
folding of the bowel called intussusception can occur causing
obstruction of the intestine and may need surgery.
When the vessels of kidneys become inflamed, they may bleed (in
about 20-35% of patients) and mild or severe hematuria and proteinuria
(protein in urine) may occur. Usually, kidney problems are not
serious. In rare cases renal disease may last for months or years
and may progress to renal failure (one to five %). In such cases,
a consultation with a nephrologist (kidney specialist) and cooperation
with the patient’s physician are necessary.
The symptoms described above usually last for about four to six
weeks. Occasionally, they may precede the appearance of skin rash
by a few days. They may appear simultaneously, or gradually in
a different order.
Other symptoms, such as seizures, brain or lung haemorrhage and
swelling of the testis, which are due to inflammation of the vessels
in other organs of the body are rarely seen.
the disease the same in every child?
The disease is more or less the same in every child, but the extent
of cutaneous and organ involvement may vary among patients. HSP
can present as one single episode, or several recurrent relapses.
the disease in children different from the disease in adults?
The disease in children is not different from the disease in adults,
but it is rarer.
is it diagnosed?
Diagnosis of HSP is mainly clinical and based on the classic purpuric
eruption, usually confined to the lower limbs and buttocks. Other
diseases that can cause a similar picture have to be excluded.
laboratory and other tests are useful?
There are no specific tests that confirm the diagnosis of HSP.
Erythrocyte sedimentation rate (ESR), or C-reactive protein (a
measure of systemic inflammation) may be normal or elevated. Blood
in stools may be positive due to intestinal haemorrhage. Urinalysis
should be performed during the course of the disease to detect
kidney involvement. Low-grade hematuria is common and resolves
A kidney biopsy is required if kidney involvement is severe (renal
insufficiency or heavy proteinuria).
it be treated?
Most HSP patients do fine and do not need any medication at all.
Treatment, when needed, is mainly supportive with control of pain,
either with simple analgesics (pain killers), or with non-steroidal
anti-inflammatory drugs such as ibuprofen and naproxen when joint
complaints are more prominent.
Administration of steroids (prednisone) is indicated in patients
with severe gastrointestinal symptoms or haemorrhage and in rare
cases with severe symptoms from other organs. If renal disease
is severe, a renal biopsy (the removal of a small part of the
effected tissue) has to be performed and, if indicated, a combined
treatment with steroids and immunosuppressive drugs is started.
are the side effects of drug therapy?
In most cases of HSP, drug treatment is not necessary, or given
only for a short time and there are no severe side effects to
be expected. In the rare cases with severe renal disease, that
require prednisone and immunosuppressive drugs for a long time,
drug side effects may be a problem (see therapy section).
long will the disease last for?
The entire course of the disease is about four to six weeks. Half
the children have at least one recurrence within six weeks, which
is usually briefer and milder than the first one. Rarely, relapses
can last longer. The majority of patients recover completely.
kinds of periodic check-ups are necessary?
Urine samples should be checked several times during the disease
and after HSP goes away in order to detect kidney problems as,
in some cases, renal involvement may occur several weeks after
the disease onset.
is the long-term outcome of the disease?
In most children the disease is self-limited and does not cause
long term problems.
The small percentage of patients presenting with persistent or
severe renal disease, may have a progressive course with possible
about school and sports?
During acute illness physical activity is usually limited, but
after recovery the child is able to go to school again and lead
a normal life. Vaccinations should also be postponed and rescheduled
by the child’s paediatrician.