Medical Author: William C. Shiel Jr., MD, FACP, FACR
Medical Editors: Dennis Lee, MD, and Melissa Conrad Stöppler, MD
Henoch-Schonlein purpura (HSP) 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 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 sometimes cramping pain in the abdomen. Henoch-Schonlein purpura is also referred to as anaphylactoid purpura.
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 that is in response to this infection (either bacteria or virus). Aside from infection, drugs can also trigger the condition. HSP occurs most commonly in children, but people of all age groups can be affected.
Classically, HSP causes skin rash, pain in the abdomen, and joint inflammation (arthritis). Not all features need be present for the diagnosis. The rash of skin lesions appears in gravity-dependent areas, such as the legs. The joints most frequently affected with pain and swelling are the ankles and the knees. Patients with HSP can develop fever. Inflammation of the blood vessels in the kidneys can cause blood and/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.
HSP is usually diagnosed based on the typical skin, joint, and kidney findings. Throat culture, urinalysis, and blood tests for inflammation and kidney function are used to suggest the diagnosis. A biopsy of the skin, and less commonly kidneys, can be used to demonstrate vasculitis. Special staining techniques (direct immunofluorescence) of the biopsy specimen can be used to document antibody deposits of IgA in the blood vessels of involved tissue.
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