Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
Age of Onset
Autosomal dominant ?A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease
Autosomal recessive ?Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype
X-linked dominant ?X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked recessive ?Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder
Mitochondrial or multigenic ?Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor ?Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Other Names (AKA)
Purpura, Schonlein-Henoch; Anaphylactoid purpura; Vascular purpura;
Blood Diseases; Kidney and Urinary Diseases; Lung Diseases
Henoch-Schonlein purpura (HSP), also called immunoglobulin A vasculitis (IgAV), is a vascular disease that primarily affects small blood vessels. The disease is characterized by abnormal deposits of immunoglobulin A (an
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
|Medical Terms||Other Names||
|80%-99% of people have these symptoms|
Pain in stomach
[ more ]
[ more ]
Death of digestive organ tissue due to poor blood supply
Blood in urine
|Nausea and vomiting||0002017|
Red or purple spots on the skin
Inflammation of blood vessel
|30%-79% of people have these symptoms|
Intermittent migraine headaches
[ more ]
[ more ]
Inflammation of testicles
Open skin sore
|5%-29% of people have these symptoms|
Inflammation of the thin layer on top of the white part of eye
Paralysis or weakness of one side of body
Flat, discolored area of skin
High urine protein levels
Protein in urine
[ more ]
Renal failure in adulthood
[ more ]
|Restrictive ventilatory defect||
Stiff lung or chest wall causing decreased lung volume
The use of glucocorticoids (steroids) in people with HSP has been under debate. While glucocorticoids have been reported to improve symptoms (particularly gastrointestinal symptoms), it is questionable whether they improve the course of the disease, and they may have potentially dangerous side effects (such as masking signs of fever and pain). They generally are not recommended as a means of preventing kidney or gastrointestinal complications.
Hospitalization may be needed for those with dehydration, debilitating abdominal or joint pain (limiting the ability to move around), significant gastrointestinal bleeding, or kidney disease. People with significant kidney involvement or advanced kidney disease may require immunosuppressive medications, hemodialysis, or kidney transplantation. While immunosuppressive therapies such as rituximab have shown promising results in initial studies, more studies are needed to determine their safety and effectiveness in people with HSP who have severe kidney involvement and other severe symptoms.
Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.
Conditions with similar signs and symptoms from Orphanet
Differential diagnoses include other causes of purpura such as thrombopenia, hemopathy or infectious diseases. In adults, Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, systemic lupus erythematosus, and mixed cryoglobulinemia (see these terms) should also be considered in the differential diagnosis.
Visit the Orphanet disease page for more information.
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
P.O. Box 28660
Kansas City, MO 64188
American Autoimmune Related Diseases Association (AARDA)
19176 Hall Road, Suite 130
Clinton Township, MI 48038
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
- The American Academy of Family Physicians provides a basic overview of Henoch-Schonlein purpura.
- MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
- The National Kidney and Urologic Diseases Information Clearinghouse (NIDDK) conducts and supports research on a broad spectrum of diseases affecting public health. Click on the link to view information on this topic.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
- Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Henoch-Schonlein purpura. Click on the link to view a sample search on this topic.
- Lopez-Mejias R, Castaneda S, Genre F, et al. Genetics of immunoglobulin-A vasculitis (Henoch-Schönlein purpura): An updated review. Autoimmun Rev. March, 2018; 17(3):301-315. https://www.ncbi.nlm.nih.gov/pubmed/29353097.
- Starkebaum GA. Henoch-Schonlein purpura. MedlinePlus. April 24, 2017; https://www.nlm.nih.gov/medlineplus/ency/article/000425.htm.
- Henoch-Schonlein Purpura. National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). September, 2012; https://www.niddk.nih.gov/health-information/kidney-disease/henoch-schonlein-purpura.
- Henoch-Schönlein Purpura. National Organization for Rare Disorders (NORD). 2015; https://rarediseases.org/rare-diseases/henoch-schonlein-purpura/.
- Scheinfeld NS. Henoch-Schonlein Purpura. Medscape Reference. January 9, 2018; https://emedicine.medscape.com/article/984105-overview.
- Heineke MH, Ballering AV, Jamin A, Ben Mkaddem S, Monteiro RC, Van Egmond M. . New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schönlein purpura). Autoimmun Rev. December, 2017; 16(12):1246-1253. https://www.ncbi.nlm.nih.gov/pubmed/29037908.
- Nicoara O, Twombley K. Immunoglobulin A Nephropathy and Immunoglobulin A Vasculitis. Pediatr Clin North Am. February, 2019; 66(1):101-110. https://www.ncbi.nlm.nih.gov/pubmed/30454736.
- Dedeoglu F, Kim S. IgA vasculitis (Henoch-Schönlein purpura): Management. UpToDate. Waltham, MA: UpToDate; October 16, 2017; https://www.uptodate.com/contents/iga-vasculitis-henoch-schonlein-purpura-management.
- González-Gay MA, López-Mejías R, Pina T, Blanco R, Castañeda S. IgA Vasculitis: Genetics and Clinical and Therapeutic Management. Curr Rheumatol Rep. April 2, 2018; 20(5):24. https://www.ncbi.nlm.nih.gov/pubmed/29611051.