Henoch-Schönlein Purpura - NORD (National Organization for Rare Disorders) (2022)

Henoch-Schönlein Purpura

NORD gratefully acknowledges the Platelet Disorder Support Association for assistance in the preparation of this report.

Synonyms of Henoch-Schönlein Purpura

  • Allergic Purpura
  • Allergic Vasculitis
  • Anaphylactoid Purpura
  • Hemorrhagic Capillary Toxicosis
  • HSP
  • Leukocytoclastic Vasculitis
  • Nonthrombocytopenic Idiopathic Purpura
  • Peliosis Rheumatica
  • Rheumatic Purpura
  • Schonlein-Henoch Purpura
  • vascular purpura

Subdivisions of Henoch-Schönlein Purpura

  • Henoch's Purpura
  • Schonlein's Purpura

General Discussion

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).

Signs & Symptoms

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.

(Video) Henoch-Schonlein Purpura: Visual Explanation for Students

Causes

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.

Affected Populations

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).

Related Disorders

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.

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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.)

Diagnosis

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.

Standard Therapies

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.

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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.

Investigational Therapies

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:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [emailprotected]

For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

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.

NORD Member Organizations

  • Platelet Disorder Support Association
    • 8751 Brecksville Road
    • Suite 150
    • Cleveland, OH 44141 USA
    • Phone: (440) 746-9003
    • Toll-free: (877) 528-3538
    • Email: [emailprotected]
    • Website: http://www.pdsa.org
  • Vasculitis Foundation

Other Organizations

  • American Kidney Fund, Inc.
    • 11921 Rockville Pike
    • Suite 300
    • Rockville, MD 20852 USA
    • Toll-free: (800) 638-8299
    • Email: [emailprotected]
    • Website: http://www.kidneyfund.org
  • Autoimmune Association
    • 19176 Hall Road, Suite 130
    • Clinton Township, MI 48038 USA
    • Phone: (586) 776-3900
    • Toll-free: (888) 852-3456
    • Email: [emailprotected]
    • Website: https://autoimmune.org
  • Genetic and Rare Diseases (GARD) Information Center
  • Henoch-Schönlein Purpura
      • 104 West 40th Street
      • Suite 500
      • New York, NY 10018
      • Phone: (212) 629-9770
      • Toll-free: (800) 633-6628
      • Email: [emailprotected]
      • Website: http://www.kidneyurology.org
    • National Kidney Foundation
      • 30 East 33rd Street
      • New York, NY 10016
      • Phone: (212) 889-2210
      • Toll-free: (800) 622-9010
      • Email: [emailprotected]
      • Website: http://www.kidney.org
    • NIH/National Heart, Lung and Blood Institute
    • NIH/National Institute of Allergy and Infectious Diseases
      • NIAID Office of Communications and Government Relations
      • 5601 Fishers Lane, MSC 9806
      • Bethesda, MD 20892-9806
      • Phone: (301) 496-5717
      • Toll-free: (866) 284-4107
      • Email: [emailprotected]
      • Website: http://www.niaid.nih.gov/

    References

    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.

    Years Published

    1987, 1989, 1997, 2005, 2015

    The information in NORD’s Rare Disease Database is for educational purposes only and is not intended to replace the advice of a physician or other qualified medical professional.

    The content of the website and databases of the National Organization for Rare Disorders (NORD) is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD. Individuals may print one hard copy of an individual disease for personal use, provided that content is unmodified and includes NORD’s copyright.

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    FAQs

    What is Henoch-Schönlein purpura caused by? ›

    Nearly half the people who have Henoch-Schonlein purpura developed it after an upper respiratory infection, such as a cold. Other triggers include chickenpox, strep throat, measles, hepatitis, certain medications, food, insect bites and exposure to cold weather.

    Is HSP a serious illness? ›

    Henoch-Schönlein purpura (HSP) affects the blood vessels and causes a spotty rash. It's not usually serious, but can sometimes lead to kidney problems.

    What is Henoch-Schönlein purpura symptoms? ›

    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).

    How long does Henoch-Schönlein purpura last in adults? ›

    Adults with HSP are more likely to have more severe disease compared to children. HSP usually ends after four to six weeks -- sometimes with recurrence of symptoms over this period, but with no long-term consequences (recurrences are fairly common).

    Can Covid vaccine trigger HSP? ›

    HSP has previously been reported following immunization with various vaccines, mostly within 12 weeks post vaccination [3]. The aim of this report is to highlight a possible association between COVID-19 vaccination (Pfizer‐BioNTech BNT16B2b2 mRNA vaccine) and first onset of HSP in a previously well adult.

    Is HSP disease curable? ›

    Henoch-Schonlein purpura usually goes away on its own within a month with no lasting ill effects. Rest, plenty of fluids and over-the-counter pain relievers may help with symptoms.

    Is HSP a mental illness? ›

    HSP isn't a disorder or a condition, but rather a personality trait that's also known as sensory-processing sensitivity (SPS).

    Can HSP fall in love? ›

    When highly sensitive people (HSPs) confide about love, there is notable depth and intensity. They fall in love hard and they work hard on their close relationships. Yes, sometimes non-HSPs sound similarly enthralled and confused by love, but on the average, HSPs have a more soul-shaking underlying experience.

    Does HSP run in families? ›

    HSP does not usually run in families. If one of your children has this condition, it is unlikely that another of your children or another family member will get it. It is not contagious, and cannot be passed on to other people.

    Is HSP a kidney disease? ›

    Henoch-Schönlein purpura (HSP), also known as anaphylactoid purpura, causes small blood vessels in your body, including those in the skin, stomach and kidneys, to become swollen and leak. One of the most obvious and common signs of this disease is a rash on the skin that looks like purple or red dots.

    Does HSP show up in a blood test? ›

    Diagnosing HSP

    There is not a specific blood test to diagnose HSP. An abdominal ultrasound may be done in patients with severe abdominal pain. In patients who have partial or unusual symptoms, a biopsy of the skin or the kidney may help make the diagnosis.

    What medications cause purpura? ›

    Drug-induced thrombocytopenic purpura is a skin condition result from a low platelet count due to drug-induced anti-platelet antibodies caused by drugs such as heparin, sulfonamines, digoxin, quinine, and quinidine.

    How do you prevent Henoch Schonlein Purpura? ›

    Can Henoch-Schönlein purpura be prevented or avoided? Doctors do not know how to prevent HSP. The disease is more common in boys than in girls. Children 2 to 11 years of age are more likely to be affected, but adults can get it too.

    Does HSP affect the immune system? ›

    HSP is an autoimmune disorder. This is when the body's immune system attacks the body's own cells and organs. With HSP, this immune response may be caused by an upper respiratory tract infection. Other immune triggers may include an allergic reaction, medicine, injury, or being out in cold weather.

    How long does it take for HSP rash to go away? ›

    This rash usually goes away in about a week, but sometimes it can last as long as one month. Abdominal pain – Belly pain tends to come and go. Most often, it will go away on its own. But if the pain is severe, worsening, or coming in waves, it could mean a more serious problem called intussusception.

    Can Covid cause Henoch-Schonlein purpura? ›

    An upper respiratory tract infection by SARS-CoV-2 could be a triggering factor in the emergence of Henoch-Schonlein purpura. Ruling out a prior infection with SARS-CoV-2 in paediatric patients presenting with what clinically appears as Henoch-Schonlein purpura should be considered.

    Can HSP affect the lungs? ›

    HSP usually affects the small blood vessels in the skin, causing a rash that is called purpura. It can also affect blood vessels in the intestines and the kidneys. HSP can lead to complications in the lungs or brain, but this is extremely rare.

    Is HSP life long? ›

    Most children with HSP fully recover within a month and have no long-term problems. Some kids who have HSP get it again, usually a few months after the first episode. If it does come back, it's usually less severe than the first episode.

    What is the best treatment for purpura? ›

    How is purpura treated?
    • Corticosteroids. Your doctor may start you on a corticosteroid medication, which can help increase your platelet count by decreasing the activity of your immune system. ...
    • Intravenous immunoglobulin. ...
    • Other drug therapies. ...
    • Splenectomy.

    Do HSP have anger issues? ›

    Due to traits of their personality, heightened empathy or childhood conditioning, many highly sensitive people have repressed anger, and do not know how to deal with their emotions healthily.

    How does HSP affect the brain? ›

    Highly sensitive people activate brain areas to a greater extent to interpret in great depth and detail the information of the affective and emotional states of the people around them, especially those close to them.

    Are HSP intelligent? ›

    HSPs are typically highly intelligent, and seek out opportunities to do deep work. Many HSPs are academics, artists, researchers, scientists and technicians with high level proficiency.

    What personality trait is HSP? ›

    A highly sensitive person (HSP) is someone who is thought to have an increased or deeper central nervous system sensitivity to physical, emotional, or social stimuli. 1 Some refer to this as having sensory processing sensitivity, or SPS for short.

    Do HSP have friends? ›

    Healthy HSP friendships deeply support each other, using listening as a two-way street. HSPs often put others' needs above their own, doing whatever they can to help a friend feel better. A highly sensitive person needs friends who appreciate their care, won't abuse it, and are happy to reciprocate.

    Are you born an HSP? ›

    High sensitivity, by definition, is a genetic trait; you're either born with it or you're not, although your early childhood experiences do play a role in shaping your level of sensitivity.

    Is HSP considered a disability? ›

    The prognosis for individuals with HSP varies Some individuals are very disabled and others have only mild disability. The majority of individuals with uncomplicated HSP have a normal life expectancy.

    Is HSP more sensitive with age? ›

    How Do HSPs Age? For better and worse, we do become even more sensitive. We are bothered by gross levels of stimulation more, process everything even more deeply, and are even more aware of consequences. But I think generally we age extremely well.

    Can HSP marry? ›

    Being married to a Highly Sensitive Person means you too can experience natural pleasures in life more easily, often, and fully. 2. Quirkville: HSPs are more easily overwhelmed by external events and may therefore at times feel broken.

    Can HSP cause heart problems? ›

    It is important to note that, while cardiac complications are rarely considered in HSP, there have been approximately 20 published cases of HSP with non-coronary artery cardiac involvement. Interestingly, not all patients had cardiac symptoms, and patients with HSP-related cardiac involvement are generally adults.

    Can you exercise with Henoch Schonlein Purpura? ›

    Take regular exercise. People with HSP lose a lot of muscle strength during illness. Ask for advice about sensible regular exercise. Eat well.

    What vitamins help with purpura? ›

    Studies have also shown that vitamin C supplementation improved purpura in patients who had a prior vitamin C deficiency. If you have purpura and are bothered by the appearance of them there are a few things that may help the lesions fade more quickly.

    Can purpura be cured? ›

    Bruises usually come back throughout the person's life, and there is no known cure for senile purpura. People who are uncomfortable with the appearance of senile purpura may contact a dermatologist for recommendations to heal and replenish the damaged skin.

    Is purpura caused by stress? ›

    Psychogenic purpura (also referred to as Gardner-Diamond syndrome, autoerythrocyte sensitization, or painful bruising syndrome) is a rare and poorly understood clinical presentation in which patients develop unexplained painful bruises, mostly on the extremities and/or face, during times of stress.

    What is the most common primary symptom of schönlein Henoch purpura? ›

    The most common sign of Henoch-Schönlein purpura is a purplish rash, which looks like bruising. It happens in all cases of HSP, usually on the lower legs and buttocks. It also can appear on the arms, face, and trunk.

    Is HSP caused by a virus? ›

    The cause of HSP is unknown, but it is most commonly triggered by a viral illness (e.g. a cold), and it occurs most often in the spring. HSP usually affects children from two to 10 years of age, but it can happen in anyone. HSP itself is not contagious, but the infection that has triggered HSP may be contagious.

    How do you prevent Henoch-Schönlein purpura? ›

    Can Henoch-Schönlein purpura be prevented or avoided? Doctors do not know how to prevent HSP. The disease is more common in boys than in girls. Children 2 to 11 years of age are more likely to be affected, but adults can get it too.

    How long does it take for Henoch-Schönlein purpura to go away? ›

    For about two-thirds of children, all symptoms will be gone in two to four weeks. For the last one-third of children, symptoms are gone in one to three months. Serious complications of HSP are rare, but it is very important to follow up with your child's doctor.

    What medications cause HSP? ›

    Propylthiouracil is the most common drug-causing vasculitis. Other drugs implicated are vancomycin, minocycline, adalimumab, rituximab, and clozapine.

    How do you test for Henoch-Schonlein purpura? ›

    There is no specific test to diagnose HSP. It is diagnosed based on recognition of the classic symptoms, and exclusion of other conditions that can cause a similar rash. In many children with a classic rash, minimal testing is needed to establish a diagnosis of HSP.

    Can Covid cause Henoch Schonlein Purpura? ›

    An upper respiratory tract infection by SARS-CoV-2 could be a triggering factor in the emergence of Henoch-Schonlein purpura. Ruling out a prior infection with SARS-CoV-2 in paediatric patients presenting with what clinically appears as Henoch-Schonlein purpura should be considered.

    Can HSP cause kidney damage? ›

    Most people recover fully from HSP without complications, but in time it can lead to chronic kidney disease and kidney failure.

    Is Henoch Schonlein Purpura lifelong? ›

    It occurs most commonly in children ages 2-6, although it can occur at any age. Patients with Henoch-Schönlein purpura develop a characteristic bruise-like rash on their arms and/or legs. In most cases, the condition does not require treatment; it will resolve on its own and the child will recover completely.

    What kind of doctor do you see for purpura? ›

    Generally, haematologists are the specialists that treat purpura.

    What is the fastest way to cure purpura? ›

    How is purpura treated?
    1. Corticosteroids. Your doctor may start you on a corticosteroid medication, which can help increase your platelet count by decreasing the activity of your immune system. ...
    2. Intravenous immunoglobulin. ...
    3. Other drug therapies. ...
    4. Splenectomy.

    Is there a blood test for HSP? ›

    There is not a specific blood test to diagnose HSP. An abdominal ultrasound may be done in patients with severe abdominal pain. In patients who have partial or unusual symptoms, a biopsy of the skin or the kidney may help make the diagnosis.

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