Henoch-Schönlein purpura (HSP) is a vasculitis (inflammation of blood vessels) that affects small blood vessels mainly in the skin, intestines, and kidneys. Symptoms can begin in children, most commonly between the ages of 4 and 7 years, soon after an upper respiratory tract infection or a streptococcalpharyngitis (sore throat infection). Children may develop arthritis (inflammation of the joints), leading to pain. A rash may start as hive-like spots (urticaria) or small raised red spots (erythematous maculopapules) on the legs and buttocks. Eventually these spots blend to form bigger areas of bruising (purpura) in the skin. Children may also develop abdominal pain that can be quite severe. Children younger than 2 years with HSP are more likely to develop edema (swelling of various areas of their bodies), which is a result of leaky small blood vessels in the skin. Kidney involvement can also cause edema, hematuria (visible or microscopic blood in the urine), or proteinuria (protein in the urine).
The signs and symptoms of HSP may mimic other serious illnesses. Therefore, a doctor may obtain tests to check for other conditions, such as an infection (suggested by the rash), thrombocytopenia (low number of platelet cells, suggested by bruising), or intussusception (telescoping of the small intestine on itself, suggested by intense abdominal pain in a young child). Tests may include
Blood tests to check the number of white blood cells (infection-fighting cells), platelets (cells that form clots), and the presence of any bacteria
Abdominal ultrasound to look for intussusception
Urinalysis to check for hematuria and proteinuria
Stool samples to check for blood in the gastrointestinal tract
Treatment is usually supportive. Unlike for other vasculitis diseases, corticosteroids (medications used to reduce inflammation) have not been shown to change the disease course. However, corticosteroids may shorten the length of severe abdominal pain.
Children with HSP may be hospitalized if their abdominal pain is too severe to control at home, if they have gastrointestinal bleeding, if joint pain prevents them from walking, or if their kidney function worsens with a change in urine output.
Although their overt symptoms usually last for 3 to 12 weeks, children diagnosed as having HSP need to be followed up by their doctor even after their symptoms have resolved. Pediatricians may ask to see these children for 3 to 6 months after their initial symptoms, more often at first. Pediatricians will check children's urine for hematuria and proteinuria until these results are normal and stable. They may also check blood pressure at these visits, since this can be affected by HSP.
Cleveland Clinic my.clevelandclinic.org/disorders/henoch_schonlein_purpura
Mayo Clinic www.mayoclinic.com/health/henoch-schonlein-purpura/DS00838
Pediatric Rheumatology International Trials Organization (PRINTO) http://www.printo.it/pediatric-rheumatology/information/UK/7.htm
To find this and previous JAMA Patient Pages, go to the Patient Page link on JAMA 's Web site at www.jama.com. Many are available in English and Spanish. A Patient Page on vasculitis was published in the August 8, 2007, issue.
Sources: Cleveland Clinic, Mayo Clinic
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Punnoose AR, Lynm C, Golub RM. Henoch-Schönlein Purpura. JAMA. 2012;307(7):742. doi:10.1001/jama.2012.81