MEDICAL CONDITIONS
Henoch-Schonlein Purpura (HSP)
or
Anaphylactoid Purpura
What is HSP?
Henoch-Schonlein purpura (HSP or anaphylactoid purpura) 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 arterial vessels called capillaries in the skin and frequently the kidneys. HSP results in skin rash (most prominent over the buttocks and behind the lower extremities associated with joint inflammation (arthritis) and cramping pain in the abdomen.
What causes HSP?
HSP occurs most often in the spring and frequently follows an infection of the throat or breathing passages. HSP seems to represent an unusual reaction of the body's immune system which is in response to this infection (either bacteria or virus). HSP occurs most commonly in children, but persons of all age groups can be affected.
What are symptoms of HSP?
Classically HSP causes skin rash, pain in the abdomen, and arthritis. Patients with HSP can develop fever. The joints most frequently affected with pain and swelling are the ankles and the knees. Inflammation of the blood vessels in the kidneys can cause blood or protein in the urine. Serious kidney complications are infrequent, but can occur.
Symptoms usually last approximately a month. Recurrences are not frequent but do occur.
What is the treatment for HSP?
While HSP is generally a mild illness that resolves spontaneously, it can cause serious problems in the kidneys and bowels. The rash can be very prominent. The treatment of HSP is directed toward the most significant area of involvement. Joint pain can be relieved by antiinflammatory medications, such as aspirin or ibuprofen. Some patients can require cortisone medications, such as prednisone, especially those with significant abdominal pain or kidney disease. With more severe kidney disease, cyclophosphamide (Cytoxan) has been used to suppress the immune system. Infection, if present, can require antibiotics.
What are the consequences of HSP?
The prognosis for patients with HSP is generally excellent. Nearly all patients have no long-term problems. The kidney is the most serious organ involved when it is affected. Rarely, patients can have serious long-term kidney damage or an abnormal bowel folding called intussusception. A few patients have recurrences of symptoms for a couple of years after the onset of the illness.
Recent data shows that HSP in adults is generally more severe than in children. Adults have more severe kidney involvement and can require more aggressive treatment. The ultimate outcome, however, is usually very good for both adults and children.
Author: William C. Shiel Jr., MD, FACP, FACR
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