Henoch-Schönlein purpura is a rare disease in which the smallest blood vessels (the capillaries) swell and become tender. The inflammation itself then causes changes in the blood vessels. It is the most common form of childhood vascular inflammation (vasculitis).
Henoch-Schönlein Purpura causes blood vessels to become inflamed (irritated and swollen). This inflammation is called vasculitis. It usually affects the small blood vessels in the skin causing a rash that is called purpura. It can also affect blood vessels in the intestines and the kidneys.
The cause of HSP is unknown. It might be triggered by a bacterial or viral infection, medicines, insect bites, vaccinations or exposure to chemicals or cold weather. It occurs most often in the spring, usually after an upper respiratory tract infection such as a cold. HSP mostly affects children, but can affect adults. Sometimes it follows a throat or chest infection. It affects boys and girls equally. Half the children affected are under the age of five. Kidney involvement is more likely to be severe in older children and adults.
Sometimes HSP is occasionally also called Berger’s disease but this should not be confused with Buerger’s disease which is a different type of vasculitis
HSP usually affects children from two to 10 years of age, but it can happen in anyone. HSP itself is not contagious. Doctors don’t know how to prevent HSP yet.
Causes
Henoch-Schönlein purpura is caused by an abnormal immune system response in which the body’s immune system attacks the body’s own cells and organs. Usually, the immune system makes antibodies, or proteins, to protect the body from foreign substances such as bacteria or viruses. In HSP, these antibodies attack the blood vessels. The factors that cause this immune system response are not known. However, in 30 to 50 percent of cases, people have an upper respiratory tract infection, such as a cold, before getting HSP.2 HSP has also been associated with
- Infectious agents such as chickenpox, measles, hepatitis, and HIV viruses
- Medications
- Foods
- Insect bites
- Exposure to cold weather
- Trauma
Genetics may increase the risk of HSP, as it has occurred in different members of the same family, including in twins.
Risk Factors
Factors that may increase the risk of developing Henoch-Schonlein purpura include –
- Age – The disease affects primarily children and young adults with the majority of cases occurring in children between 2 and 6 years of age.
- Sex – Henoch-Schonlein purpura is slightly more common in boys than girls.
- Race – White and Asian children are more likely to develop Henoch-Schonlein purpura than black children are.
- Time of year – Henoch-Schonlein purpura strikes mainly in autumn, winter and spring but rarely in summer.
Symptoms
The symptoms usually begin suddenly and may include –
- Bloody stools
- Fever
- Headache
- Joint pain
- Loss of appetite
- Painful menstruation
- Red or purple spots on the skin. These usually appear on the buttocks, lower legs and elbows
- Stomach cramps, nausea, vomiting or diarrhea
The disease can affect the joints, kidneys, digestive system and – in rare cases – the brain and spinal cord. There are different forms of the disease, which affect different parts of the body. For example –
- Schönlein’s purpura affects the skin and joints but not the digestive system
- Henoch’s purpura causes spots on the skin and severe abdominal problems. Persons with this form of the disease do not have joint symptoms.
Complications
Complications associated with Henoch-Schonlein purpura include –
Kidney damage – The most serious complication of Henoch-Schonlein purpura is kidney damage. Occasionally the damage is severe enough that dialysis or a kidney transplant may be needed.
Bowel obstruction – In rare cases, Henoch-Schonlein purpura can cause intussusception — a condition in which a section of the bowel folds into itself like a telescope, which prevents matter from moving through the bowel.
Treatment
Symptoms of HSP usually last for about a month. Most of the time, it goes away on its own without treatment. To help your child feel better, the doctor may recommend certain medications, such as –
- Antibiotics to treat the causative infection, if applicable
- Painkillers (such as acetaminophen)
- Anti-inflammatory medicines (such as ibuprofen) to relieve joint pain and inflammation
- Corticosteroids (such as prednisone) for severe abdominal pain or kidney disease
HSP usually gets better on its own without causing lasting problems. About half of people who have had HSP once will get it again. A few people will have kidney damage because of HSP. This may occur in the first week or so of illness, but there may be a delay of weeks or months before it appears. Your child’s doctor will want to test urine samples and blood pressure several times after the HSP goes away to check for kidney problems. These checks should go on for at least six months and some doctors recommend a blood pressure and urine check every year for life.
Alternative Treatment
Foot Bath – This therapy combines traditional Chinese herbs with the warm foot bath. The herbs we choose are based on the patient’s specific condition. The effective ingredients can stimulate the meridians on feet, thus the whole blood vessels can be unlocked. More over, they have the function of fighting against inflammation in blood vessels, thus treating HSP from the root.
Immunotherapy – There are six steps of this therapy. They inhibit the inflammations and restore normal immune system. It is also involve in TCM. Since most TCM come from nature, and these remedies are external application, they are very safe for children.
Hot Compress Therapy – This is an external application of Chinese herb medicine. According to patient’s specific disease condition, kidney doctors would choose the herbs and blend them together sufficiently. During the treatment process, we compress the powder of the herbs on kidney area, transmitting the medicine ingredients to the renal lesion by electromagnetic wave.
Reference –
http://familydoctor.org/familydoctor/en/diseases-conditions/henoch-schonlein-purpura.html
http://umm.edu/health/medical/ency/articles/henochschonlein-purpura
http://pedsinreview.aappublications.org/content/35/10/447
https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/henoch-schonlein-purpura
https://www.cedars-sinai.edu/Patients/Health-Conditions/Henoch-Schonlein-Purpura.aspx
http://bestpractice.bmj.com/best-practice/monograph/110.html
http://www.gosh.nhs.uk/medical-information-0/search-medical-conditions/henoch-sch-nlein-purpura-hsp
http://www.dermnetnz.org/vascular/hsp.html
http://www.vasculitis.org.uk/about-vasculitis/henoch-schonlein-purpura
http://patient.info/doctor/henoch-schonlein-purpura-pro
http://www.vasculitisfoundation.org/education/forms/henoch-schonlein-purpura/