Rheumatic purpura, otherwise known as Henoch-Schönlein purpura (HSP) is an unusual inflammatory disease of the small blood vessels and is commonly a self-limited disease. It is the most common form of childhood vascular disease and results in some inflammatory changes among small vessels.
Symptoms of HSP usually starts unexpectedly, including headache, fever, cramping, hives, loss of appetite, bloody diarrhea, painful menstruation, and joint pain. HSP can evolve from the kidneys, joints, or the digestive system, but, in rare cases, it grows into the brain and spinal cord.
Schönlein’s purpura is a form of the disorder that affects the joints and skin but not the gastrointestinal tract. Individuals with this disorder have purplish spots on the skin and acute abdominal problems. The cause of HSP is unknown, although research demonstrates that it is related to an abnormal reaction of the immune system.
People with rheumatic purpura usually have small red or purple spots on the skin, especially in the legs. Purpura spots are caused by small hemorrhages under the skin and are not related to the irregular low levels of platelets. Other bruises related to HSP include large hives or ulcers. Due to the abnormal fluid buildup in the soft tissues, swelling can show up in the face and neck. In unusual cases, difficulty in breathing may occur due to swelling and edema in the throat.
Some people with HSP have problems with kidney function, blood in the urine and inflammatory changes in the kidneys. Some people may even develop severe kidney problems.
The assessment of HSP may be tough, especially in adults. The disease is always confused with the other forms of vascular inflammation. Regular laboratory tests are not reliable to diagnose the disorder. The platelet count is usually normal but white blood cell and erythrocyte sedimentation rate levels may be elevated.
The disorder is diagnosed by the appearance of a combination of visible skin bruises and joint weakness, along with a positive test for blood in the urine and a skin biopsy that reveals inflammation of the arterial and venous capillaries.
When evidence of streptococcal infection is present, antibiotic therapy is prescribed. Mild childhood cases of the disease often improve spontaneously with advanced age. There is no specific treatment, however, in most patients, the disease has a limited course and the outlook for recovery is good.