Henoch-Schönlein purpura
Immunoglobulin A vasculitis; Leukocytoclastic vasculitis; HSP
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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.
Definition
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).
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.
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)
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
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.
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.
Media items
Henoch-Schonlein purpura on the lower legs
Henoch-Schonlein purpura
Henoch-Schonlein purpura
Henoch-Schonlein purpura
Henoch-Schonlein purpura on an infant's foot
Henoch-Schonlein purpura on an infant's legs
Henoch-Schonlein purpura on an infant's legs
Henoch-Schonlein purpura on the legs
Related articles
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
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
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.
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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