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Henoch-Schönlein purpura

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).

Alternative Names

Immunoglobulin A vasculitis; Leukocytoclastic vasculitis; HSP

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:

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.

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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