Henoch-Schonlein Purpura (HSP), is quite difficult to pronounce right; pronounce it as hen-awk sher-line purr-purr-ah. HSP is a disease that affects the blood vessels and causes blood vessels in the skin to swell and bleed. Inflammation of the blood vessels is called vasculitis. When the blood vessels bleed under the skin they cause purple rashes on the skin called purpura. HSP is commonly found as rashes on the buttocks and legs, however, it can occur in the bowel and kidney also resulting in bleeding of the intestine and kidney. HSP occurs commonly in children of age 2-11 years but it can affect anyone at any time. HSP occurs twice as often in boys than in girls.
Causes
Although an aura of mystery surrounds the exact cause of HSP, researchers know that HSP occurs when the person’s immune system does not function normally as it should. An immune reaction is triggered when a protein called Immunoglobulin A (Ig A) is deposited on the blood vessels triggering an immune reaction and causing the blood vessels to swell and bleed. HSP may occur after a child has a bacterial or viral infection of the upper respiratory tract. If a child has an upper respiratory tract infection, the body’s immune system is awakened naturally to fight against the germs. Once the entire germs are gone, the immune system should rest, but with HSP, the immune system continues to attack other cells of the body causing it to swell and bleed. Apart from these, HSP can also occur as a reaction to certain foods, medicines, vaccinations, and insect bites. HSP is not contagious.
Symptoms
HSP is a multisystem disorder that mainly affects the skin, kidneys, gastrointestinal tract, and joints, although it can very rarely affect other organs. Symptoms of HSP last only for 4-6 weeks, fortunately with no longer serious complications. In a few people, symptoms may come and go, and 1 in 3 people suffer persistent recurrence. Recurrence occurs within a few months and the consecutive episodes of HSP are milder than the initial attack.
Rashes and bruises or red spots may appear on the skin, buttocks, legs, and back of the arms; rarely the bruises may spread to the upper part of the body. There could be abdominal pain along with nausea, vomiting, diarrhoea, and sometimes blood in stools. HSP may also manifest as arthritis, pain and swelling in joints, usually in the knee and ankle and very rarely in the elbow and wrist. HSP may damage the kidney, and there could be the presence of blood in urine (haematuria). Haematuria usually goes away without any permanent kidney damage, but protein in urine and high blood pressure may suggest more serious kidney problems. Other symptoms include headache, fever, and painful menstruation in women.
Complications
HSP in a few cases result in serious complications such as
- Kidney failure
- Bowel bleeding
- Intussusception of bowel (invagination of one part of bowel/intestine into another, blocking the bowel)
- HSP during childhood in women may result in high blood pressure during pregnancy
Diagnosis
HSP can be easily diagnosed by the presence of rashes, joint pain, and abdominal pain, but sometimes these symptoms are caused by other conditions, which causes a delay in diagnosing HSP. Therefore, the physician may perform other tests to confirm HSP. Confirming HSP requires a series of tests including a urine sample collected from the patient and checked for blood and protein in the urine. An increased level of protein in the urine indicates severe kidney damage.
Blood tests may be required. Blood with increased levels of urea, nitrogen, and creatinine indicates that the kidneys are not functioning properly. Healthy kidneys usually filter urea and creatinine from blood.
A skin biopsy may be done. A small sample of skin is taken and checked under a microscope. If it shows the presence of a large number of white blood cells and IgA then HSP is confirmed.
Biopsy of the kidney is taken very rarely. The sample is examined under a microscope. Biopsy also helps to decide which medicine is effective in treating kidney disease.
X-ray or ultrasound scanning of the abdomen will be done to check whether appendicitis, bowel tear or obstruction is not the cause of abdominal pain.
Treatment
Even if HSP last for a few months, it can be cured completely. There is no specific treatment for HSP, suggested treatments aim at minimizing the symptoms such as abdominal pain, joint pain, and swelling. Treating rashes and joint pain is not required in most people as these symptoms fade away after 4-6 weeks. But to help children feel better doctors may recommend certain treatment procedures. Many patients go for over-the-counter medicine like acetaminophen to help reduce pain. If the patient has severe arthritis then the doctor may prescribe a steroid called Prednisone, or anti-inflammatory drugs to reduce pain and swelling. In case of severe bowel pain or intestinal bleeding, the doctor may go on with Prednisone or very rarely surgery may be required to correct the problem. In a few cases, it might damage the kidney and result in complete kidney failure. If the kidney damage is permanent then the patient might require dialysis or kidney transplantation. Doctors may also prescribe drugs to suppress the immune system. These immunosuppressive drugs may prevent further damage to the kidney and prevent kidney damage from progressing into permanent kidney failure.
After treatment
The prognosis of HSP is excellent. HSP usually resolves on its own, and many people do not require any treatment. Parents need not worry too much about their kids suffering from HSP, as it does not result in any serious complications most of the time. About 1 in 3 kids may have a recurrence, and the severity of recurrence is milder than the first HSP episode. The outlook after treatment is considered very well until and unless kidney complications develop. Once diagnosed with HSP it is always better to have kids checked frequently for their kidney function along with blood and urine tests.
Conclusion
The best way to deal with HSP is to allow the disease to run its course. Complete bed rest and giving kids few pain relievers can help them from joint pain and swelling. Following the doctor’s instructions is very important in case the child develops kidney complications, but fortunately, it is very rare in children. Parents need not go by the complicated name of the disease, HSP is curable and usually complications are very rare.
