Henoch-Schönlein purpura (HSP) is a rare inflammatory disease of the small blood vessels (capillaries) and is usually a self-limited disease. It is the most common form of childhood vascular inflammation (vasculitis) and results in inflammatory changes in the small blood vessels. The symptoms of HSP usually begin suddenly and may include headache, fever, loss of appetite, cramping, abdominal pain, painful menstruation, hives, bloody diarrhea, and joint pain. Red or purple spots typically appear on the skin (petechiae). Inflammatory changes associated with HSP can also develop in the joints, kidneys, digestive system, and, in rare cases, the brain and spinal cord (central nervous system).
In one form of the disorder, termed Schönlein's purpura, the skin and joints are affected but the gastrointestinal tract is not. In another form, known as Henoch's purpura, affected individuals have purplish spots on the skin and acute abdominal problems, such as glomerulonephritis (a type of kidney disorder). People with Henoch's purpura are not affected by joint disease.
The exact cause of HSP is not fully understood, although research demonstrates that it is related to an abnormal response by the immune system or, in some rare cases, an extreme allergic reaction to certain offending substances (e.g., foods or drugs).
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