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IgA vasculitis - Henoch-Schonlein purpura

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

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).

Images

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

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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:

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:

When to Contact a Medical Professional

Contact your provider if:

Related Information

Glomerulonephritis
Hypersensitivity vasculitis
Immune response
Renal

References

Dinulos JGH. Hypersensitivity syndromes and vasculitis. In: Dinulos JGH, ed. Habif's Clinical Dermatology. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 18.

Stone JH. The systemic vasculitides. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 249.

Stone JH. Immune complex–mediated small vessel vasculitis. In: Firestein GS, McInnes IB, Koretzky GA, Mikuls TR, Neogi T, O'Dell JR, eds. Firestein & Kelley's Textbook of Rheumatology. 12th ed. Philadelphia, PA: Elsevier; 2025:chap 92.

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 pubmed.ncbi.nlm.nih.gov/29136340/.

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Review Date: 4/1/2025  

Reviewed By: Diane M. Horowitz, MD, Rheumatology and Internal Medicine, Northwell Health, Great Neck, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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