Henoch-Schönlein Purpura - NORD (National Organization for Rare Disorders) (2023)

The symptoms of HSP 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 bloody diarrhea. Joint pain (arthralgia) may develop in any joint of the body, especially the knees and ankles. Some people with HSP experience vomiting and diarrhea; others may have severe constipation and unusually dark stool (melena).

Individuals with HSP 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 is common with some other forms of purpura. Other skin lesions associated with HSP 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 HSP have problems with kidney function, such as glomerulonephritis, in which the portion of the kidney that separates waste from the blood is damaged. 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 HSP 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 HSP 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.

HSP 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).

Common purpura is the most prevalent type of purpura, occurring most often in women over age 50. When there has been no injury, purpura lesions occur more often than subcutaneous bleeding. However, following surgery or even minor injuries, blood vessel fragility results in excessive bleeding. The bleeding may be reduced by short-term corticosteroid therapy and/or, in postmenopausal women, the administration of estrogen.

Scurvy, a type of purpura, results from a deficiency of vitamin C in the diet. Symptoms may include experience generalized weakness, anemia, spongy gums, and a tendency to bleed (hemorrhage) under the skin (subcutaneous) and from the delicate mucous membranes that line the mouth and the gastrointestinal tract. Scurvy rarely occurs in modern civilizations due to improvement of diet and availability of foods that contain Vitamin C.

Gardener-Diamond syndrome (Autoerytrocyte Sensitization) is a rare disorder that is sometimes called painful bruising syndrome. It is characterized by purpura spots, mainly in young women. Gardener-Diamond syndrome is thought to be an autoimmune disorder.

Vasculitis (angiitis) is a vascular inflammatory disease that may occur alone or in association with other allergic or rheumatic diseases. Inflammation of the vascular walls constricts the blood vessels and may cause a lack of blood supply to certain areas of the body (ischemia), tissue loss (necrosis), and/or blood clots (thrombosis). Any size vessel or any part of the vascular system may be affected, and symptoms are relative to the system involved. Symptoms may include fever, headache, profound loss of appetite, weight loss, weakness, abdominal pain, diarrhea, and/or muscle and joint pain. Since there are many forms of Vasculitis, there are many causes. Some types may be caused by allergic reactions or hypersensitivity to certain medications such as sulfur drugs, penicillin, propylthiouracil, other drugs, toxins, and other inhaled environmental irritants. Other forms may occur because of a fungal infection, parasites or viral infections, while in some cases there may be no apparent cause. (For more information on this disorder, choose “Vasculitis” as your search term in the Rare Disease Database.)

Cutaneous necrotizing vasculitis is a relatively common inflammatory disease of the blood vessels, including the veins, arteries, and smaller blood vessels (capillaries). This disorder typically affects the skin and may occur alone or in conjunction with allergic, infectious, or rheumatic diseases. Symptoms may include skin nodules, small hemorrhages under the skin, and/or skin lesions that may be red and flat (macules). These may develop on many parts of the body, especially the back, hands, buttocks, and/or legs. In some cases, hives that are intensely itchy (urticaria) or ring-shaped ulcers may also be present. Fever, generalized discomfort (malaise), and/or muscle or joint pain may also occur. The exact cause of cutaneous necrotizing vasculitis is unknown. Some lesions may be caused by an allergic reaction or hypersensitivity to certain medications such as sulfa or penicillin, other drugs, toxins, and inhaled environmental irritants.

Kawasaki disease is an inflammatory disease of childhood characterized by fever, skin rashs, swollen lymph nodes (lymphadenopathy), inflammation of the arteries (polyarteritis), and inflammation of blood vessels (vasculitis). Inflammatory changes cause destructive lesions in the blood vessels that can lead to complications involving the liver, gall bladder, and heart. The symptoms may include an abnormally high fever that begins suddenly and lasts for approximately two weeks. Other symptoms may include redness in the lining of the eyelids of both eyes (bilateral conjunctivitis), irritability, fatigue, redness (inflammation) of the mouth and tongue (stomatitis), cracking of the lips, swelling of the lymph nodes in the neck (cervical adenopathy), and/or skin rashs. The exact cause of Kawasaki disease is not fully understood. It may be related to two previously unknown strains of staphylococcus and streptococcus bacteria and/or a possible immunological abnormality. (For more information on this disorder, choose “Kawasaki” as your search term in the Rare Disease Database.)

Immune thrombocytopenia is a rare platelet disorder characterized by unexplained low levels of platelets in the circulating blood. Symptoms may include nosebleeds (epistaxis), small red or purple spots on the skin that represent small hemorrhages under the skin (petechiae), and/or bleeding from the rectum and/or urinary tract. Anemia may follow and produce weakness and fatigue. Other people with this disorder may experience episodes of elevated fever and abnormal enlargement of the spleen. No specific cause for Immune thrombocyopenia has been identified. Current evidence supports an immunologic basis, since most patients have antiplatelet antibodies that are identifiable. (For more information on this disorder, choose “Immune Thrombocytopenia” as your search term in the Rare Disease Database.)

Thrombotic thrombocytopenic purpura (TTP) is a rare blood disease characterized by abnormally low levels of circulating platelets in the blood, abnormal destruction of red blood cells, kidney dysfunction, and disturbances of the nervous system. Symptoms of this disorder, which begin suddenly, may include fever, headache, purpura spots on the skin and mucous membranes, joint pain (arthralgias), partial loss of feeling in the arms or legs (paresis), changes in mental status, and/or seizures. The exact cause of TTP is not known. It may be due to an infectious agent or to an autoimmune reaction. (For more information on this disorder, choose “Thrombotic Thrombocytopenia Purpura” as your search term in the Rare Disease Database.)

The diagnosis of HSP 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 (urinalysis), and a skin biopsy that shows inflammation of the arterial and venous capillaries.

Therapy

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. There is no specific treatment, however, 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 HSP 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.

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

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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 (IVIG) has been used on an experimental basis to treat some children with severe abdominal pain associated with HSP. Further research is needed to determine the long-term safety and effectiveness of immunoglobulins for the treatment of this disorder.

TEXTBOOKS
Saulsbury FT. Henoch-Schönlein Purpura. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003.

REVIEW ARTICLES
Ballinger S. Henoch-Schönlein Purpura. Curr Opin Rheumatol. 2003;15:591-94.

Crowson AN, Mihm MC Jr, Magro CM. Cutaneous vasculitis: a review. J Cutan Pathol. 2003;30:161-73.

Ozen S. The spectrum of vasculitis in children. Best Pract Res Clin Rheumatol. 2002;16:411-25.

Nadeau SE. Neurologic manifestations of systemic vasculitis. Neurol Clin. 2002;20:123-50.

Davin JC, Weening JJ. Henoch-Schönlein Purpura nephritis: an update. Eur J Pediatr. 2001;160:689-95.

Rostoker G. Schönlein-henoch purpura in children and adults: diagnosis, pathophysiology and management. Biodrugs. 2001;15:99-138.

Saulsbury FT. Henoch-Schönlein purpura. Curr Opin Rheumatol. 2001;13:35-40.

INTERNET
Clowse MEB. Henoch-Schönlein purpura. MedlinePlus. Medical Encyclopedia. Update Date4/20/2013. https://www.nlm.nih.gov/medlineplus/ency/article/000425.htm Accessed October 21, 2015.