Brief overview
- Prognosis: Usually good, healing on its own after a few days to weeks, rarely relapses, in case of organ involvement rarely late sequelae up to definite kidney failure possible
- Symptoms: Small hemorrhages of the skin, especially on the lower legs; if joints or organs are involved (rare): Symptoms ranging from joint inflammation to neurological problems depending on the organ affected
- Causes and risk factors: Autoimmune disease in which excess IgA antibodies lead to vascular inflammation; infections and medications are under discussion as triggers, exact cause unknown to date
- Diagnosis: medical history, physical examination, visual diagnosis based on typical symptoms, examinations of blood, urine, stool, and ultrasound or magnetic resonance imaging in cases of suspected organ involvement
- Treatment: Usually not necessary, in some cases painkillers or anti-inflammatory drugs, severe courses are treated with ACE inhibitors, antihypertensive drugs or surgery (for example in case of testicular torsion or intestinal obstruction)
What is Schönlein-Henoch purpura (in children)?
The frequency is given as 15 to 25 affected persons per 100,000 children and adolescents. Boys are affected more frequently than girls. Only very rarely are adults affected, but then usually more severely.
In Schönlein-Henoch purpura, the small vessels of the skin, joints, gastrointestinal tract and kidneys are mainly affected. The disease often occurs after a preceding upper respiratory tract infection or due to other triggers such as medications. The blood vessels become more permeable over time as a result of the inflammation, causing pinpoint hemorrhages (petechiae) in the skin.
In addition, swelling usually occurs on the backs of the feet and hands and in the joints. Children with Schönlein-Henoch purpura suddenly no longer want to walk. The children also often suffer from abdominal pain, vomiting and diarrhea. In some cases, purpura Schönlein-Henoch results in inflammation of the kidneys (glomerulonephritis).
The disease begins acutely and progresses in episodes. As a rule, it heals without serious consequences.
Schönlein-Henoch purpura was named after the physicians Johann Lukas Schönlein and Eduard Heinrich Henoch.
Course of the disease and prognosis
In most cases, Schönlein-Henoch purpura heals on its own. The duration of the disease ranges from three days to about two months. On average, purpura is cured after about twelve days. It then progresses in episodes of varying intensity. However, there are also courses that last up to two years or, in very rare cases, become chronic.
In most cases, the disease remains without further consequences – but there are also possible late effects, especially if organs are involved.
In some cases, relapses occur after a period without symptoms.
What late effects are possible?
In a severe course, it is possible that skin and soft tissue necroses (dying tissue parts) form, which heal spontaneously with scarring. The healing process then often takes between four and six weeks.
In very rare cases, Purpura Schönlein-Henoch leads to definitive (terminal) renal failure. In such a very rare case, patients are dependent on dialysis or even kidney transplantation.
A late sequelae often occurs much later. For example, women who once had IgA vasculitis in childhood are more likely to develop kidney problems during pregnancy.
What are the symptoms?
In Schönlein-Henoch purpura, small skin hemorrhages (petechiae) are prominent. In some cases, the internal organs are affected, usually accompanied by mild fever. Onset is usually sudden. Children complain of symptoms such as headache, loss of appetite, and crampy abdominal pain. The symptoms occur in episodes.
The leading symptoms of Schönlein-Henoch purpura affect different parts of the body:
Skin
The skin lesions are very different. Often, Schönlein-Henoch purpura begins as single skin hemorrhages with a diameter of one to three millimeters, which then coalesce and appear as extensive bleeding. Usually, the skin hemorrhages occur symmetrically and do not itch.
In children under two years of age, a different variant of Schönlein-Henoch purpura is seen in some cases. It is called “acute infantile hemorrhagic edema” or “Seidlmayer cocard purpura.” In this case, the typical skin hemorrhages are found on the arms and legs as well as on the facial skin.
Joints
About 65 percent of children with Schönlein-Henoch purpura show a sudden onset of painful swelling and restriction of movement, especially in the ankle and knee joints (purpura rheumatica). Usually both sides of the body are affected. Parents then notice that their child “suddenly doesn’t want to walk”.
Gastrointestinal tract
Kidney
After one to two weeks, it is possible that there will be visible or invisible blood in the urine (macro- or microhematuria). This affects at least 30 percent of children with Schönlein-Henoch purpura. Protein excretion in the urine (proteinuria), elevated blood pressure and renal dysfunction are also possible. Such kidney involvement is referred to as Schönlein-Henoch nephritis.
As a complication, it is very rare for renal involvement to lead to definitive (terminal) renal failure.
Central nervous system
Very rarely, the cerebral vessels are affected in Schönlein-Henoch purpura. Headache, behavioral disturbances, seizures, paralysis, and impaired consciousness are then possible. Cerebral hemorrhage is a very rare possible complication.
Testis
Rarely, Purpura Schönlein-Henoch results in testicular inflammation (orchitis): the testicles hurt and are swollen. It is important to rule out testicular torsion (rotation of the testis and spermatic cord about the longitudinal axis), as this may lead to infertility if left untreated.
Complications
Another complication that rarely occurs in Schönlein-Henoch purpura is intussusception (intussusception).
In Schönlein-Henoch nephritis, renal disease sometimes recurs later. In this case, kidney function worsens.
Women who have previously had Schönlein-Henoch purpura are more prone to kidney problems during pregnancy.
Causes and risk factors
The exact cause of Schönlein-Henoch purpura is unknown. Nearly 80 percent of cases are thought to be due to drug, viral and bacterial triggers. Presumably, there is an immunologic reaction following upper respiratory tract infections such as those with influenza A viruses (flu agents) or β-hemolytic streptococci.
Most drugs are known to trigger Schönlein-Henoch purpura under certain circumstances, but especially antibiotics, anti-inflammatory drugs (anti-inflammatory drugs such as cortisone and non-steroidal anti-inflammatory drugs) and drugs that promote water excretion (thiazides).
Vascular inflammation
The antibodies, which are so-called immunoglobulins A (IgA), are deposited on the vessel walls in Schönlein-Henoch purpura. IgA immune complexes are formed, which trigger an inflammatory reaction (complement activation), causing the destruction of small vessels not only in the skin, but also in the gastrointestinal tract and kidneys.
In Schönlein-Henoch purpura, the cells that produce IgA proliferate in excessive numbers. IgA are antibodies that usually form a first defense against many pathogens.
The vascular damage results in blood leaking into the surrounding connective tissue, causing the typical hemorrhage. An inflammatory reaction of a blood vessel is technically called vasculitis. It is also called type III allergy (Arthus reaction).
Is Schönlein-Henoch purpura contagious?
Since Purpura Schönlein-Henoch is an autoimmune inflammation of the vessels, this disease is not contagious. No precautions need to be observed.
Examinations and diagnosis
The physician will make his diagnosis based on the characteristic symptoms as well as other examination methods and laboratory values.
Medical history and physical examination
There is no specific laboratory value for Purpura Schönlein-Henoch that allows a definite diagnosis. However, the physician diagnoses the disease in other ways. To do this, he or she first obtains the history of the disease (anamnesis). Possible questions the pediatrician may ask are:
- How long has your child had pinpoint skin bleeding?
- Does your child have joint pain and/or fever?
- Has your child had a recent cold?
- Have you noticed movement restrictions during play or sports?
- Does your child complain of abdominal pain or nausea?
- Have you noticed blood in your child’s stool or urine?
- Does your child have diarrhea?
This is followed by a physical examination. The pediatrician pays particular attention to the characteristic skin appearance of Schönlein-Henoch purpura. A skin biopsy is only necessary if the findings are unclear. If the typical appearance of a Purpura Schönlein-Henoch is shown, this is not necessary. A histological examination may confirm the presence of Schönlein-Henoch purpura.
In a blood sample from the child, the physician determines the inflammation parameters such as erythrocyte sedimentation rate and C-reactive protein. These are usually only slightly elevated in Schönlein-Henoch purpura.
In addition, the coagulation factors should be determined to see whether there may not be a deficiency of coagulation factor XIII, since this causes a tendency to bleeding.
To rule out other forms of vascular inflammation, blood tests for immunoglobulins (Ig), antinuclear antibodies (ANA), and antineutrophil cytoplasmic antibodies (ANCA) are performed. In Schönlein-Henoch purpura, ANA and ANCA are negative.
Urine and kidney tests
Urinalysis may provide evidence of renal involvement. Elevated levels of protein (proteinuria) and red blood cells (hematuria) may indicate glomerulonephritis.
If there are signs of kidney involvement over a long period of time, or if the affected child’s kidney function deteriorates rapidly, the doctor will take a tissue sample from the kidney (kidney biopsy).
Stool examination
Ultrasound
Ultrasound examination for abdominal pain is used to detect intestinal wall bleeding and whether intussusception may be present. In addition, the physician uses ultrasound to examine the affected joint, kidneys and, in boys, testicles.
Examination of the brain
If it is suspected that the central nervous system is affected by the vasculitis, a magnetic resonance imaging (MRI) scan of the head is usually performed.
Exclusion diagnostics
Based on the clinical examination and laboratory results, the physician will attempt to rule out other conditions such as coagulation disorders, sepsis, septic arthritis, and other forms of vasculitis.
Treatment
In most cases, the symptoms of children with Schönlein-Henoch purpura improve on their own and no therapy is necessary. Hospitalization is advisable only in cases of complicated course with abdominal pain, fever, painful joint complaints, poor general condition, and in children under two years of age and adults (a severe course is possible).
Treatment of severe Schönlein-Henoch purpura
In the case of kidney involvement, the so-called creatinine value in the urine provides information about the severity of Schönlein-Henoch nephritis. Creatinine is a breakdown product of creatine, which serves as an energy reserve in the muscles.
If creatinine levels are slightly elevated for more than six weeks (less than two grams of creatinine per gram of urine: < 2 g/g creatinine), doctors often use certain medications - ACE inhibitors or an angiotensin-1(-AT-1) receptor antagonist. If creatinine is more elevated (> 2g/g), high-dose cortisone medications may be considered. These are given for about twelve weeks, with the dosage being gradually reduced again in the final weeks (“tapering”).
Increased blood pressure is possible in connection with kidney involvement. In this case, the doctor will adjust your child’s blood pressure to normal with medication. In addition, he will recommend that you have your child’s kidney function checked regularly for up to two years after Schönlein-Henoch nephritis.
