Henoch-Schönlein purpura 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 Henoch-Schönlein purpura usually begin suddenly and may include headache, fever, loss of appetite, cramping abdominal pain, and joint pain. Red or purple spots typically appear on the skin (petechial purpura). Inflammatory changes associated with Henoch-Schönlein purpura 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. People with Henoch's purpura are not affected by joint disease.
The exact cause of Henoh-Schönlein purpura is not fully understood, although research suggests that it may be an autoimmune disease or, in some rare cases, an extreme allergic reaction to certain offending substances (e.g., foods or drugs).
The symptoms of Henoch- Schönlein purpura usually begin suddenly. In addition to the characteristic red spotting of the skin (most often on the buttocks and backs of the legs), they may include headache, loss of appetite, and/or fever. The skin typically becomes red (diffuse erythema). Cramping abdominal pain may occur and is usually most severe during the night. Blood may be present in the stool and abnormal bleeding (hemorrhaging) from the gastrointestinal tract can cause bloodly diarrhea. Joint pain (arthralgia) may develop in any joint of the body, especially the knees and ankles. Some people with Henoch-Schönlein purpura experience vomiting and diarrhea; others may have severe constipation and unusually dark stools (melena).
Individuals with Henoch-Schönlein purpura typically develop small red or purple spots (petechiae) on the skin, especially on the legs. These purpura spots are caused by small hemorrhages under the skin and are not associated with abnormally low levels of platelets (nonthrombocytopenic) as with some other forms of purupura. Other skin lesions associated with Henoch-Schönlein purpura include large hives (urticarial wheals) or ulcers (necrotic), especially on the buttocks and legs. Swelling may occur in the face and neck due to abnormal fluid accumulation in the soft tissues of these areas (angioneurotic edema). In rare cases, swelling and edema in the throat can cause breathing difficulties that can lead to life-threatening respiratory problems.
Between one-quarter and one-half of people with Henoch-Schönlein purpura have problems with kidney function. Blood in the urine (hematuria) and inflammatory changes in the kidneys may also develop. Some people may develop severe kidney disease, including IgG nephropathy, chronic inflammation of the kidneys (nephritis), and/or nephrotic syndrome leading to kidney failure.
In rare cases, a portion of the affected person’s bowel or intestine may fold in upon itself (intussusception). This can result in substantial pain and, if conservative measures do not resolve the problem, surgery may be required.
When the central nervous system is involved, individuals with this disorder may experience severe headaches, perceptual changes, convulsions, visual difficulties (optic atrophy), and/or seizures.
The exact cause of Henoch-Schönlein purpura is not known, although research suggests that this disease may be caused by immune system dysfunction (i.e., increased IgA immune complexes). Autoimmune disorders are caused when the body’s natural defenses against “foreign” or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons.
In some cases, it has been suggested that this disorder may be an extreme allergic reaction to certain foods, such as chocolate, milk, eggs, or beans. Various drugs (e.g., nifedipine, diltiazem, cefuroxime, diclofenac, etc.), bacteria (e.g., Streptococcus), and insect bites have also been indicated as possible causes in some cases. Rubella precedes the first symptoms of Henoch-Schönlein purpura in about 30 percent of cases. In about 66 percent of cases, an upper respiratory infection precedes the onset of symptoms by about 1 to 3 weeks. A definite link to viral infections has not been proven.
Henoch-Schönlein purpura is a rare disorder that affects more males than females. The disease may occur in all age groups, although it most commonly affects children.
In children, the initial symptoms typically begin after the age of 2 years and usually last for about 4 weeks and the disease usually has a somewhat mild course. About 50 percent of affected children experience one or more recurrences, usually within months. The rate of recurrence seems to be higher among those children whose initial disease was more severe.
Most affected children have been between 2 and 11 years of age. In the USA, about 14 cases occur per 100,000 school-aged children. It is generally a benign (non-threatening) disorder appears in most instances to cure itself (self-limiting).
The diagnosis of Henoch-Schönlein purpura may be difficult, especially in adults. The disease is frequently confused with other forms of vascular inflammation (see Related Disorders section of this report). Routine laboratory tests are usually not definitive for the disorder. The platelet count is typically normal although white blood cell and sedimentation rates may be elevated.
The disorder is diagnosed by a combination of the presence of skin lesions and/or joint tenderness, combined with a confirmed test for blood in the urine, and a skin biopsy that shows inflammation of the arterial and venous capillaries.
If individuals are thought to have HSP as the result of an allergic reaction, they must strictly avoid the offending substance (e.g., food or drug). When evidence of streptococcal infection is present, antibiotic therapy is prescribed. Mild childhood cases of the disease often improve spontaneously with advancing age. In most patients, the disease has a limited course and the outlook for recovery is good.
If non-steroid anti-inflammatories fail to relieve symptoms, some patients may be treated with glucocorticoids (steroid) drugs such as prednisone. These drugs may be useful to help control acute abdominal and joint pain. In some cases, swelling of soft tissues (angioedema) may be helped with steroid drugs. Dapsone may be prescribed when prednisone is contra-indicated or fails to relieve symptoms. The use of steroids to treat this disorder remains a matter of controversy in the medical literature. Some research indicates that steroids do not shorten the length of the illness or reduce the frequency or recurrence of symptoms. Other studies indicate that early steroid treatment may help to reduce the risk of kidney damage.
Patients with Henoch-Schönlein purpura who have advanced kidney disease and renal failure will probably benefit from mechanical cleansing of the waste products from the blood (hemodialysis). Aggressive and supportive care may be necessary during acute kidney crisis. Some patients with severe kidney disease have undergone kidney transplantation. However, the disease can recur in the transplanted kidney. Other treatment is symptomatic and supportive.
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Experimental treatment with a combination drug therapy of anticoagulants (i.e., heparin and acenocoumarol), corticosteroids, and immunosuppressants has been tested on adults with severe cases of HSP. Further studies are needed to determine the long-term safety and effectiveness of this form of therapy for the treatment of this disorder.
Plasmapheresis as a means of removing unwanted substances (toxins, metabolic substances, and plasma parts) from the blood has also been tried experimentally. Blood is removed from the patient, and blood cells are separated from plasma. The patient’s plasma is then replaced with other human plasma and the blood is transfused back into the patient. This therapy is still under investigation to analyze side effects and effectiveness. More research is needed before plasmapheresis can be recommended for use in all but the most severe cases of HSP.
Intravenous immunoglobulin (IVGG) has been used on an experimental basis to treat some children with severe abdominal pain associated with Henoch-Schönlein purpura. Further research is needed to determine the long-term safety and effectiveness of immunoglobulins for the treatment of this disorder.
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