Helping you to be a WellOne.


 
E-mail Form
Email Results

 
 
Print-Friendly
Bookmarks
bookmarks-menu

IgA vasculitis - Henoch-Schönlein purpura

Immunoglobulin A vasculitis; Leukocytoclastic vasculitis; Henoch-Schönlein purpura; HSP

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

Causes

IgA vasculitis 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 IgA vasculitis may include:

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.
  • Complete blood count. The platelet could should be normal.
  • Coagulation tests: these should be normal.
  • Skin biopsy, especially in adults.
  • Blood tests to look for other causes of blood vessel inflammation, such as systemic lupus erythematosus, ANCA-associated vasculitis or hepatitis.
  • In adults, a kidney biopsy should be done.
  • Imaging tests of the abdomen if pain is present.

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 IgA vasculitis have only one episode. One third of children have more episodes. People should have close medical follow-up for 6 months after episodes to look for signs of kidney disease. Adults have a greater risk of developing chronic 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 IgA vasculitis, and they last for more than a few days.
  • You have colored urine or low urine output after an episode.

References

Arntfield RT, Hicks CM. Systemic lupus erythematosus and the vasculitides. In: Walls RM, Hockberger RS, Gausche-Hill M, eds. Rosen's Emergency Medicine: Concepts and Clinical Practice. 9th ed. Philadelphia, PA: Elsevier; 2018:chap 108.

Dinulos JGH. Hypersensitivity syndromes and vasculitis. In: Habif TP, Dinulos JGH, Chapman MS, Zug KA, eds. Skin Disease: Diagnosis and Treatment. 4th ed. Philadelphia, PA: Elsevier; 2018:chap 11.

Feehally J, FLoege J. Immunoglobulin A nephropathy and IgA vasculitis (Henoch-Schönlein purpura). In: Feehally J, Floege J, Tonelli M, Johnson RJ, eds. Comprehensive Clinical Nephrology. 6th ed. Philadelphia, PA: Elsevier; 2019:chap 23.

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.

Lu S, Liu D, Xiao J, et al. Comparison between adults and children with Henoch-Schönlein purpura nephritis. Pediatr Nephrol. 2015;30(5):791-796. PMID: 25481021 www.ncbi.nlm.nih.gov/pubmed/25481021.

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

Sunderkötter CH, Zelger B, Chen KR, et al. Nomenclature of cutaneous vasculitis: Dermatologic addendum to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheumatol. 2018;70(2):171-184. PMID: 29136340 www.ncbi.nlm.nih.gov/pubmed/29136340.

  • 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


     

    Review Date: 4/8/2019

    Reviewed By: Gordon A. Starkebaum, MD, MACR, ABIM Board Certified in Rheumatology, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

    The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
    adam.com

     
     
     

     

     

    A.D.A.M. content is best viewed in IE9 or above, Firefox and Google Chrome browser.
    Content is best viewed in IE9 or above, Firefox and Google Chrome browser.