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Henoch-Schönlein purpura

Immunoglobulin A vasculitis; Leukocytoclastic vasculitis; HSP

 

Henoch-Schönlein purpura (HSP) is a disease that involves purple spots on the skin, joint pain, gastrointestinal problems, and glomerulonephritis (a type of kidney disorder).

Causes

 

HSP is caused by an abnormal response of the immune system. The result is inflammation in the microscopic blood vessels in the skin. Blood vessels in the joints, kidneys, or the intestines may also be affected. It is unclear why this occurs.

The syndrome is mostly seen in children between ages 3 and 15 years, but it may be seen in adults. It is more common in boys than in girls. Many people who develop this disease had an upper respiratory infection in the weeks before.

 

Symptoms

 

Symptoms and features of HSP may include:

  • Purple spots on the skin (purpura). This occurs in nearly all children with HSP. This most often occurs over the buttocks, lower legs, and elbows.
  • Abdominal pain
  • Joint pain
  • Abnormal urine (may have no symptoms)
  • Diarrhea, sometimes bloody
  • Hives or angioedema
  • Nausea and vomiting
  • Swelling and pain in the scrotum of boys
  • Headache

 

Exams and Tests

 

The health care provider will look at your body and look at your skin. The physical exam will show skin sores (purpura, lesions) and joint tenderness.

Tests may include:

  • Urinalysis should be done in all cases.
  • Skin biopsy, especially in adults.
  • Blood tests to look for other causes of blood vessel inflammation, such as systemic lupus erythematosus or hepatitis.
  • In adults, a kidney biopsy should be done.

 

Treatment

 

There is no specific treatment. Most cases go away on their own. Joint pain may improve with NSAIDS such as naproxen. If symptoms do not go away, you may be prescribed a corticosteroid medicine such as prednisone.

 

Outlook (Prognosis)

 

The disease most often gets better on its own. Two thirds of children with HSP have only one episode. One third of children have more episodes. People should have close medical follow-up for 6 months after HSP to look for signs of kidney disease. 

 

Possible Complications

 

Complications may include:

  • Bleeding inside the body
  • Blocking of the intestine (in children)
  • Kidney problems (in rare cases)

 

When to Contact a Medical Professional

 

Call your provider if:

  • You develop symptoms of HSP, and they last for more than a few days.
  • You have colored urine or low urine output after an episode of HSP.

 

 

References

Ferguson JM, Tammaro D. Henoch-Schönlein purpura. In: Ferri FF, eds. Ferri's Clinical Advisor 2017. Philadelphia, PA: Elsevier; 2017:562.e1-563.e1.

Hahn D, Hodson EM, Willis NS, Craig JC. Interventions for preventing and treating kidney disease in Henoch-Schönlein purpura (HSP). Cochrane Database Syst Rev. 2015;(8):CD005128. PMID: 26258874 www.ncbi.nlm.nih.gov/pubmed/ 26258874.

Horowitz R. Pediatric abdominal disorders. In: Adams JG, ed. Emergency Medicine. 2nd ed. Philadelphia, PA: Elsevier Saunders; 2013:chap 22.

Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11. PMID: 23045170 www.ncbi.nlm.nih.gov/pubmed/23045170.

Patterson JW. The vasculopathic reaction pattern. In: Patterson JW, ed. Weedon's Skin Pathology. 4th ed. Philadelphia, PA: Elsevier Churchill Livingstone; 2016:chap 8.

Van Why SK, Avner ED. Henoch-Schönlein purpura nephritis. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 515.

Text only

 
  • Henoch-Schonlein purpura on the lower legs

    Henoch-Schonlein purpura on the lower legs - illustration

    Henoch-Schonlein Purpura: This disorder usually presents with red to purple bumps on the legs, often accompanied by aching in the joints and fever. This condition follows an infection and usually resolves without treatment. Skin lesions most commonly occur below the knee but may also be seen on the thigh, buttocks, and rarely on the arms.

    Henoch-Schonlein purpura on the lower legs

    illustration

  • Henoch-Schonlein purpura

    Henoch-Schonlein purpura - illustration

    Henoch-Schonlein is a type of hypersensitivity vasculitis and inflammatory response within the blood vessel. It is caused by an abnormal response of the immune system. The exact cause for this disorder is unknown.

    Henoch-Schonlein purpura

    illustration

  • Henoch-Schonlein purpura

    Henoch-Schonlein purpura - illustration

    The syndrome is usually seen in children, but people of any age may be affected. It is more common in boys than in girls. Many people with Henoch-Schonlein purpura had an upper respiratory illness in the previous weeks.

    Henoch-Schonlein purpura

    illustration

  • Henoch-Schonlein purpura

    Henoch-Schonlein purpura - illustration

    There is no specific treatment for Henoch-Schonlein purpura. Most cases resolve spontaneously without treatment. If symptoms persist, therapy with corticosteroids such as prednisone is usually tried.

    Henoch-Schonlein purpura

    illustration

  • Henoch-Schonlein purpura on an infant's foot

    Henoch-Schonlein purpura on an infant's foot - illustration

    Henoch-Schonlein purpura is more commonly seen in children than adults and often occurs after an upper respiratory infection. It causes skin rashes that bleed into the skin (petechiae and purpura). Bleeding may also occur from the gastrointestinal tract and kidneys.

    Henoch-Schonlein purpura on an infant's foot

    illustration

  • Henoch-Schonlein purpura on an infant's legs

    Henoch-Schonlein purpura on an infant's legs - illustration

    Henoch-Schonlein purpura is more commonly seen in children than adults and often occurs after an upper respiratory infection. It causes skin rashes that bleed into the skin (petechiae and purpura). Bleeding may also occur from the gastrointestinal tract and kidneys.

    Henoch-Schonlein purpura on an infant's legs

    illustration

  • Henoch-Schonlein purpura on an infant's legs

    Henoch-Schonlein purpura on an infant's legs - illustration

    Henoch-Schonlein purpura is more commonly seen in children than adults and often occurs after an upper respiratory infection. It causes skin rashes that bleed into the skin (petechiae and purpura). Bleeding may also occur from the gastrointestinal tract and kidneys.

    Henoch-Schonlein purpura on an infant's legs

    illustration

  • Henoch-Schonlein purpura on the legs

    Henoch-Schonlein purpura on the legs - illustration

    Henoch-Schonlein purpura is more commonly seen in children than adults and often occurs after an upper respiratory infection. It causes skin rashes that bleed into the skin (petechiae and purpura). Bleeding may also occur from the gastrointestinal tract and kidneys.

    Henoch-Schonlein purpura on the legs

    illustration

    • Henoch-Schonlein purpura on the lower legs

      Henoch-Schonlein purpura on the lower legs - illustration

      Henoch-Schonlein Purpura: This disorder usually presents with red to purple bumps on the legs, often accompanied by aching in the joints and fever. This condition follows an infection and usually resolves without treatment. Skin lesions most commonly occur below the knee but may also be seen on the thigh, buttocks, and rarely on the arms.

      Henoch-Schonlein purpura on the lower legs

      illustration

    • Henoch-Schonlein purpura

      Henoch-Schonlein purpura - illustration

      Henoch-Schonlein is a type of hypersensitivity vasculitis and inflammatory response within the blood vessel. It is caused by an abnormal response of the immune system. The exact cause for this disorder is unknown.

      Henoch-Schonlein purpura

      illustration

    • Henoch-Schonlein purpura

      Henoch-Schonlein purpura - illustration

      The syndrome is usually seen in children, but people of any age may be affected. It is more common in boys than in girls. Many people with Henoch-Schonlein purpura had an upper respiratory illness in the previous weeks.

      Henoch-Schonlein purpura

      illustration

    • Henoch-Schonlein purpura

      Henoch-Schonlein purpura - illustration

      There is no specific treatment for Henoch-Schonlein purpura. Most cases resolve spontaneously without treatment. If symptoms persist, therapy with corticosteroids such as prednisone is usually tried.

      Henoch-Schonlein purpura

      illustration

    • Henoch-Schonlein purpura on an infant's foot

      Henoch-Schonlein purpura on an infant's foot - illustration

      Henoch-Schonlein purpura is more commonly seen in children than adults and often occurs after an upper respiratory infection. It causes skin rashes that bleed into the skin (petechiae and purpura). Bleeding may also occur from the gastrointestinal tract and kidneys.

      Henoch-Schonlein purpura on an infant's foot

      illustration

    • Henoch-Schonlein purpura on an infant's legs

      Henoch-Schonlein purpura on an infant's legs - illustration

      Henoch-Schonlein purpura is more commonly seen in children than adults and often occurs after an upper respiratory infection. It causes skin rashes that bleed into the skin (petechiae and purpura). Bleeding may also occur from the gastrointestinal tract and kidneys.

      Henoch-Schonlein purpura on an infant's legs

      illustration

    • Henoch-Schonlein purpura on an infant's legs

      Henoch-Schonlein purpura on an infant's legs - illustration

      Henoch-Schonlein purpura is more commonly seen in children than adults and often occurs after an upper respiratory infection. It causes skin rashes that bleed into the skin (petechiae and purpura). Bleeding may also occur from the gastrointestinal tract and kidneys.

      Henoch-Schonlein purpura on an infant's legs

      illustration

    • Henoch-Schonlein purpura on the legs

      Henoch-Schonlein purpura on the legs - illustration

      Henoch-Schonlein purpura is more commonly seen in children than adults and often occurs after an upper respiratory infection. It causes skin rashes that bleed into the skin (petechiae and purpura). Bleeding may also occur from the gastrointestinal tract and kidneys.

      Henoch-Schonlein purpura on the legs

      illustration

    Tests for Henoch-Schönlein purpura

     
       

      Review Date: 4/24/2017

      Reviewed By: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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