Henoch Schonlein purpura (HSP), also called Immunoglobulin A (IgA) vasculitis, is a rare inflammatory disease that affects capillaries (small blood vessels) and causes an increased tendency to bleed.
HSP is usually a self-limiting condition that means your body resolves the issues caused by the condition without any active treatment. However, in some cases, the disease may develop severe life-threatening complications, such as:
- Kidney damage, which may require dialysis or a renal transplant
- Ischemia of the intestine or intussusception, which is a condition where one part of the intestine moves into another part of the intestine, resulting in intestinal obstruction
- Diffuse alveolar hemorrhage, which leads to acute respiratory failure
HSP may affect all ages, but children younger than 10 years are most commonly affected. The risk of developing complications is higher in adults than in children.
What are the causes of Henoch Schonlein purpura?
The exact cause of Henoch Schonlein purpura (HSP) is not clear, but it is believed to be an abnormal reaction of the immune system toward:
- An infection, such as upper respiratory tract infection caused by streptococci, hepatitis, chickenpox, and human immunodeficiency virus.
- A severe allergic reaction to food, medications, or insect bite, or cold weather.
During an event of an infection or allergy, the immune cells produce antibodies against the infection-causing agents, such as bacteria and viruses.
- These antibodies may attack healthy blood vessels and cause inflammation, bleeding secondary to damage of the blood vessel walls.
- This bleeding may result in skin rash that appears as hives and small bumps on the skin (due to hemorrhages), which appear like bruises.
HSP is sometimes seen in patients who belong to the same family and even in twins. This may suggest that HSP may have a genetic cause. However, further research is needed to prove the statement.
What are the symptoms of Henoch Schonlein purpura?
The symptoms of Henoch Schonlein purpura (HSP) may include:
- Purpura, a cutaneous rash caused by HSP (appears in the form of little bruises or reddish-purple patches on the buttocks, around the elbows, or on the legs and feet)
- Hives or angioedema
- High blood pressure
- Nausea and vomiting
- Abdominal pain
- Bloody diarrhea
- Arthritis (joint pain)
- Kidney disease
- Abnormal urine
- Swelling and pain in the scrotum in males
How is Henoch Schonlein purpura diagnosed?
The symptoms of Henoch Schonlein purpura (HSP) may be consistent with other vascular inflammatory diseases, so HSP is diagnosed by careful analysis of all possible signs and symptoms. The patients with HSP develop antibodies, which eventually get deposited in the skin.
The following tests are done to diagnose HSP:
- Blood test: The blood samples collected from the patient are checked for white blood cells count, inflammatory markers, and analysis of all agents that evaluate the kidney function. All coagulation tests are done to check for the function of clotting factors.
- Urine test: A urine test is done to check for the signs of kidney damage, such as blood or protein in the urine.
- Skin biopsy: Some skin tissue is collected under the influence of local anesthesia and is examined for the presence of antibodies that get deposited under the skin in HSP.
- Kidney biopsy: With the help of imaging techniques, a biopsy needle is used to collect some tissue from the kidneys to analyze kidney damage.
- Imaging studies: Using imaging studies, such as ultrasound and computed tomography scans, the abdominal organs and kidneys are evaluated to rule out other causes of symptoms.
What are the treatment options for Henoch Schonlein purpura?
In most cases, treatment is not necessary for Henoch Schonlein purpura, and the rash resolves within a week. There may be a recurrence of rashes over the next 6 to 16 weeks in a few patients with persistent illness.
To relieve the symptoms, the following medications are prescribed to the patients:
- Oral corticosteroids, such as prednisolone, are prescribed to patients with severe disease
- Nonsteroidal anti-inflammatory medicines, such as ibuprofen, may help alleviate joint and stomach pain
- When there is severe renal involvement, steroids are frequently coupled with stronger immunosuppressive medicines or intravenous immunoglobulin
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National Organization for Rare Disorders. Henoch-Schönlein Purpura. https://rarediseases.org/rare-diseases/henoch-schonlein-purpura/
Cedars-Sinai. Henoch-Schönlein Purpura (HSP) in Children. https://www.cedars-sinai.org/health-library/diseases-and-conditions---pediatrics/h/henoch-schonlein-purpura-hsp.html
Kids Health. Henoch-Schönlein Purpura (HSP). https://kidshealth.org/en/parents/hsp.html
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