Henoch-Schonlein Purpura Definition, Reason, Symptoms, Treatment & Prevention
Understanding
Henoch-Schonlein Purpura (HSP) is inflammation of small blood vessels. This condition of inflammation of small blood vessels is often referred to as vasculitis.
Blood vessels in various parts of the body become irritated and swell, causing rashes (bleeding on the skin), and can attack the kidneys and intestines.
This disease is rare, occurring about 14 per 100,000 population. Although it can affect any age group, HSP is more common in children under 10 years of age.
Also, HSP is usually not serious. In most cases, this condition will heal within 4–6 weeks.
However, in certain cases, inflammation can result in serious complications, such as intussusception (abnormal folds in the intestine), orchitis (swelling of the testicles), and kidney failure.
Symptom
HSP disease will show classic symptoms, such as:
- Skin rash (purpura) in the form of purplish-red spots or small bruises, usually on the buttocks, elbows, feet, face, and upper body. The rash stands out so that it will be felt when touched.
Occasionally, itching may appear in the area of the rash. This rash is found in all cases of HSP in children.
- Joint pain in the knees and ankles that may be swollen, warm, and tender to the touch. Joint symptoms are found in 60-84 percent of HSP cases.
- Digestive symptoms, such as nausea, vomiting, abdominal pain, or gastrointestinal bleeding. Often, these HSP symptoms appear before the skin rash appears. Gastrointestinal symptoms are found in 35-85 percent of cases.
- Other symptoms that are quite severe can include blood in the urine or stools, as well as vomiting and diarrhea.
- Symptoms of infection such as fever, headache, muscle aches may also appear before the rash because HSP is related to a previous infection.
- Symptoms of the kidneys can also be found, such as protein in the urine, hypertension, edema (swelling) in the eyes and ankles. This symptom is encountered in 20-50 percent of HSP patients.
- Symptoms that rarely occur (2-8 percent) are on the central nervous system, namely headaches, seizures, brain hemorrhage, paralysis of half the body, etc.
Reason
Henoch-Schonlein Purpura is caused by an immune system reaction that may arise due to a previous infection.
This infection can originate from bacteria or viruses in the throat, airways, and lungs.
HSP is not contagious and is not inherited. In rare cases, HSP is associated with typhoid vaccination, cholera, yellow fever, measles, hepatitis B, food, drugs (especially antibiotics and anti-convulsants), chemicals, insect bites, and cold weather.
Risk Factors
There are several things that can increase a person’s risk of developing HSP, namely:
- Children (90 percent of cases are found in children under 10 years)
- Male gender
- Post-infection, especially upper respiratory tract
Diagnosis
The doctor will diagnose Henoch-Schonlein Purpura (HSP) from the symptoms that arise, medical history, and physical examination.
According to the American College of Rheumatology, there are four criteria for confirming HSP. The presence of 2 of these 4 criteria confirms the diagnosis.
The criteria are as follows:
- There is a skin rash in the form of palpable purpura
- Age less than or equal to 20 years when symptoms of the disease appear
- Presence of pain over all areas of the abdomen, or diagnosed with intestinal ischemia with bloody diarrhea ( bowel angina )
- The results of a skin biopsy found granulocytes in the walls of blood vessels
Meanwhile, the European League Against Rheumatism/Pediatric Rheumatology European Society diagnoses HSP if there are mostly purpura on the lower limbs (mandatory), plus at least one of the following four criteria:
- Recent (acute) joint pain or inflammation
- New generalized abdominal pain (acute)
- There is an impact on the kidneys, in the form of protein or blood in the urine
- Tissue microscopy examination showed typical results ( leukocytoclastic vasculitis or proliferative glomerulonephritis with IgA deposits )
Investigations can be carried out to confirm the diagnosis, such as:
- Blood tests are done to check for signs of infection and kidney function
- A urine test is done to check for the presence of blood or protein in the urine
- Blood pressure test to check for the possibility of hypertension which is also associated with kidney disorders
- A skin biopsy may be done if the doctor is still unsure of the HSP diagnosis
- Ultrasound and kidney biopsy may be done if kidney problems occur
Treatment
Henoch-Schonlein Purpura (HSP) is usually not serious and self-limiting (94 percent of children and 89 percent of adults recover) in 2-6 weeks.
Patients can undergo outpatient treatment to the doctor. Rashes, joint pain, and stomach pain usually go away without treatment.
Therefore, the treatment given is not specific for HSP. HSP treatment is more supportive or to relieve the patient’s symptoms.
Patients are advised to get plenty of rest, as well as elevating the affected leg to prevent purpuric rash.
Anti-inflammatory drugs such as ibuprofen can be used to relieve joint pain. However, you are advised to be careful using it because this drug should not be consumed by those who have kidney or intestinal disorders.
For cases like this, you can use the painkiller paracetamol. Steroid-class drugs are also sometimes used to relieve abdominal pain.
Prevention
Until now it is not known with certainty the cause of inflammation of Henoch-Schonlein Purpura (HSP). Because it is quite difficult to take meaningful precautions.
When an infection occurs in a child, try to get your child to get treatment from a doctor. Also make sure the child gets good nutrition, gets enough sleep, and gets enough rest.
In addition, do ways to improve and increase your little one’s immune system.
Complications
Some of the complications of HSP that can arise are:
- On the kidney: glomerulonephritis, hemorrhagic cystitis, nephrotic syndrome, renal failure, obstruction of the renal tract
- Gastrointestinal: intussusception, intestinal perforation, intestinal infarction, gastrointestinal obstruction, gastrointestinal bleeding, pancreatitis
- In the lungs: alveoli bleeding, pulmonary effusion
- On the central nervous system: blindness, paralysis, convulsions, cerebral hemorrhage, etc
- In other body parts: inflammation of the heart (myocarditis), inflammation of the testicles (orchitis), swelling of the scrotum, twisting of the testicles (torsion testis)
When to See a Doctor?
If there is a palpable rash, especially on the lower limbs, accompanied by a child who looks less fit.