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What is Henoch-Schonlein purpura (HSP)?

m Henoch-Schonlein purpura (HSP) is a form of blood

vessel inflammation or vasculitis.

m HSP affects the small vessels called capillaries in the

skin and frequently the kidneys.

m HSP results in skinrash (most prominent over the

buttocks and behind the lower extremities) associated


with joint inflammation (arthritis) and sometimes
cramping pain in the abdomen.
What causes HSP?

m Frequently follows an infection of the throat or

breathing passages

m Drugs

(HSP occurs most commonly in children, but people of all age


groups can be affected.)
What are symptoms of HSP?

m skin rash (skin lesions appears on gravity-dependent areas


such as the legs)
m pain in the abdomen
m joint inflammation (arthritis) (ankles and knees)
m fever
m blood and/or protein in the urine (hematuria/proteinuria)
m kidney complications are infrequent but can occur

(Not all features need be present for the diagnosis. Symptoms


usually last approximately a month. Recurrences are not
frequent but do occur.)
How is HSP diagnosed?
m typical skin, joint, and kidney findings

m throat culture
m urinalysis
m blood tests for inflammation

m kidney function
m biopsy of the skin, and less commonly kidneys, can be
used to demonstrate vasculitis
m direct immunofluorescence of the biopsy specimen can be
used to document antibody deposits of IgA in the blood
vessels of involved tissue
'ypical purpura on lower legs
and buttocks
—ore severe case of HSP on
child's foot, leg, and arm
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What is the treatment for HSP?
'he treatment of HSP is directed toward the most significant area of
involvement.
m antiinflammatory medications (aspirin or ibuprofen) -for joint pains

m cortisone medications ( prednisone or prednisolone)-especially those with


significant abdominal pain or kidney disease

m immunosuppressants [Cyclophosphamide (Cytoxan), azathioprine (Imuran),


or mycophenolate mofetil (Cellcept)]- for glomerulonephritis or nephritis

m antibiotics- for infection

(While HSP is generally a mild illness that resolves spontaneously, it can


cause serious problems in the kidneys and bowels. 'he rash can be very
prominent, especially on the lower extremities).
What is the prognosis for patients
with HSP?
m 'he prognosis for patients with HSP is generally excellent.

m 'he kidney is the most serious organ involved when it is affected.

m Rarely, patients can have serious long-term kidney damage or an


abnormal bowel folding called intussusception.

m Some patients have recurrences of symptoms, particularly skin


rash, for months to a year after the onset of the illness.

m 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.

m 'he ultimate outcome is usually very good for both adults and
children.

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