Purpura Schönlein-Enoch

Purpura Schönlein-Enoch: description

Under Schönlein-Henoch purpura (anaphylactoid purpura, Vasculitites allergica) is understood to mean an inflammation of the small blood vessels and capillaries triggered by their own immune system. In technical language, such inflammation is referred to as vasculitis. Schönlein-Henoch purpura is the most common form of vasculitis in infants and schoolchildren, affecting children under 10 in 90 percent of cases. Rarely, infants and adolescents are affected.

The Schönlein-Henoch purpura mainly affects the small vessels of the skin, joints, gastrointestinal tract and kidneys. The disease often occurs after a previous upper respiratory tract infection or other triggers such as medication. The blood vessels become permeable over time due to the inflammation, causing punctate bleeding (petechiae) in the skin. In addition, swelling usually occurs on the back of the feet and the back of the hand, as well as on the joints. Children with Purplura Schönlein-Enoch suddenly do not want to walk anymore. The children also often suffer from abdominal pain, vomiting and diarrhea. In some cases, the purplish Schönlein-Henoch causes inflammation of the kidneys (glomerulonephritis).

The disease begins acutely and runs in batches. As a rule, it heals without serious consequences.

The Schönlein-Henoch Purpura was named after the German doctors Johann Lukas Schönlein and Eduard Heinrich Enoch.

Purplura Schönlein-Enoch: Symptoms

Purpura Schönlein-Henoch focuses on minor skin bleeding. However, it can also affect the internal organs, usually accompanied by a slight fever. The beginning is usually sudden. The children complain of symptoms such as headache, loss of appetite and cramping abdominal pain. The complaints occur in batches.

The guiding symptoms of a purplish Schönlein-Enoch affect different body regions:

Purpura Schönlein-Enoch: Skin

The skin is always involved in purplish Schönlein-Henoch and shows punctate, sometimes even area, merging into each other skin bleeding. The skin bleeds are palpable, but not wegdrückbar. Mostly, they occur mainly on the extensor sides of the lower leg and on the buttocks. Rarely, they are also found on the lower abdomen, on the extensor sides of the upper extremities and in boys in the testes area. The skin lesions may be different. Frequently, the Schönlein-Henoch purpura begins as a single skin bleeding with a diameter of one to three millimeters, which then flow together and appear as a planar bleeding. Most of the skin bleeding occurs symmetrically and does not itch.

In children under two years, another variant of the Schönlein-Henoch purpura may show up. It is called “acute infantile hemorrhagic edema” or 2Seidlmayer corkarpurpura “. The typical skin bleeding can be found here in the area of ​​the arms and legs as well as the facial skin.

Purpura Schönlein-Henoch: Joints

About 65 percent of children with Schönlein-Henoch purpura show a sudden onset of painful swelling and limited mobility, especially in ankle and knee joints (purpura rheumatica). Mostly both sides of the body are affected. Parents then notice that their child can “suddenly stop walking”.

Purplura Schönlein-Henoch: Gastrointestinal tract

Colic-like abdominal pain, but also bloody stool (abdominal purpura) and diarrhea can be caused by the inflammation of the small intestinal vessels. The gastrointestinal tract is affected in half of children with Schönlein-Henoch purpura. Vomiting and bloody stool may indicate invagination of the intestinal loops.

Purpura Schönlein-Henoch: kidney

After one to two weeks, there may be visible or invisible blood in the urine (macro- or microscopic hematuria). This affects at least 30 percent of children with Purplura Schönlein-Enoch. Also, protein excretion via the urine (proteinuria), increased blood pressure and a renal dysfunction are possible. In such kidney involvement one speaks of a Schönlein -Henoch nephritis.

Purpura Schönlein-Enoch: central nervous system

Very rarely, the cerebral vessels are affected in Purpura Schönlein -Hennoch. Headaches, behavioral disorders, seizures, paralysis, and confinement may occur.

Purpura Schönlein-Enoch: testicles

Rarely does it occur in Purpura Schönlein-Henoch for orchitis (orchitis): The testicles hurt and are swollen. It is important to exclude a testicular torsion (rotation of the testicle and spermatic cord around the longitudinal axis), as this untreated can lead to infertility.

Purplura Schönlein-Henoch: Complications

Complications of Purpura Schönlein-Henoch are very rare and occur only occasionally. In a so-called fulminant form suddenly appear symmetrical flat skin bleeding. Most of these bleedings develop into blisters, which eventually become necrotic (tissue destruction). In this variant of a purplish Schönlein-Enoch, the child may suffer a life-threatening shock.

Another complication that can occur in Purplica Schönlein-Enoch is the invagination of the intestine.

In the case of Schönlein-Henoch nephritis, it can later lead to a renewed disease of the kidneys. The kidney function worsens.

Women who have ever had a Purplica Schönlein-Henoch tend to be prone to kidney problems during pregnancy.

Purplura Schönlein-Henoch: causes and risk factors

The exact cause of the Schönlein-Henoch purpur is unknown. Nearly 80 percent of cases are caused by drug, viral and bacterial triggers. Presumably, there is an immunological reaction to upper respiratory tract infections such as influenza A virus (influenza A) or β-hemolytic streptococci.

Most medicines can trigger a Schönlein-Henoch purpura, but especially antibiotics, anti-inflammatories (anti-inflammatory drugs such as cortisone and non-steroidal anti-inflammatory drugs) and drugs that promote water excretion (thiazides). Other triggers such as insect bites, vaccinations, hyposensitization or certain diseases of the immune system such as connective tissue diseases (collagenosis), liver diseases and malignant tumors are possible. Sometimes a Purpura Schönlein-Henoch appears for no apparent reason.

Purplura Schönlein-Henoch: Inflammation

The antibodies, which are so-called immunoglobulins A (IgA), deposit on Purpura Schönlein-Henoch on the vessel walls. It creates IgA immune complexes that trigger an inflammatory response (complement activation) and can lead to the destruction of small vessels not only in the skin, but also in the gastrointestinal tract and in the kidneys. The vascular damage causes blood to leak into the surrounding connective tissue and trigger the typical bleeding. An inflammatory reaction of a blood vessel is called in the jargon vasculitis. It is also referred to as type III allergy (Arthus reaction).

Purpura Schönlein-Enoch: Contagious?

Since the purpura Schönlein-Enoch is an autoimmune inflammation of the vessels, this disease is not contagious. There are no precautions to be taken.

Purplura Schönlein-Henoch: examinations and diagnosis

If you suspect Purpura Schönlein-Henoch, it is advisable to introduce your child to a pediatrician. He can determine whether the rash is actually Schönlein-Henoch purpura or another disease.

characteristic symptoms and other examination methods and laboratory values.

Anamnesis and physical examination

There is no specific laboratory value for Schönlein-Henoch purpura that allows a clear diagnosis. However, the doctor can determine the disease in other ways. For this purpose, he first determines the history of the disease (anamnesis). Possible questions of the pediatrician could be:

• Since when does your child have punctiform haemorrhages?
• Does your child have joint pain and / or fever?
• Did your child recently have a cold?
• Have you noticed movement restrictions while playing or playing sports?
• Does your child complain of abdominal pain or nausea?
• Did you notice 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 appearance of the skin in Purpura Schönlein-Enoch. A skin biopsy is required only in case of unclear findings. If the typical appearance of a purplish Schönlein-Enoch shows up, this is not necessary. A histologic (histological) proof can confirm a Schönlein-Henoch purpura.

blood test

In a blood sample of the child, the inflammatory parameters are determined such as erythrocyte sedimentation rate and the C-reactive protein. These are usually only slightly increased at Purpura Schönlein-Enoch.

In addition, the coagulation factors should be determined as to whether there may not be a deficiency of coagulation factor XIII as it may cause bleeding tendencies.

To exclude other forms of vasculitis, the blood immunoglobulins (Ig), antinuclear antibodies (ANA) and antineutrophil cytoplasmic antibodies (ANCA) are studied. In Purpura Schönlein-Henoch, ANA and ANCA are negative.

Examinations of urine and kidneys

A urine test can give an indication of kidney involvement. Increased levels of protein (proteinuria) and red blood cells (hematuria) may be associated with glomerulonephritis.

If signs of kidney involvement appear over a long period of time or if the affected child rapidly experiences a deterioration in kidney function, the physician will take a kidney biopsy sample.

stool examination

If a hemorrhage in the gastrointestinal tract is suspected in a child with Purplura Schönlein-Henoch, a test for hidden (occult) blood in the stool (hemoccult test, guaiac test) may give an indication.

Ultrasonic

The ultrasound examination for abdominal pain is used to determine bowel wall bleeding and if there may be an intussusception. In addition, the affected joint, the kidneys and in boys the testicles are examined by ultrasound.

Examination of the brain

If it is suspected that the central nervous system is also affected by the vascular inflammation, an MRI (magnetic resonance imaging, MRI) of the head is usually performed.

exclusion diagnosis

On the basis of the clinical examination and the laboratory values, the doctor will try to rule out other diseases such as coagulation disorders, sepsis, septic arthritis and other forms of vascular inflammation.

Purpura Schönlein-Enoch: Treatment

Most children with Purpura Schönlein-Enoch improve their symptoms on their own and therapy is not necessary. A hospital stay is only advisable in case of complicated course with abdominal pain, fever, painful joint complaints, poor general condition and children under two years and adults (a difficult course is possible).

The symptoms can be treated well symptomatically. For pain, the doctor prescribes acetaminophen or non-steroidal anti-inflammatory drugs such as ibuprofen or naproxen. If the intestine is affected, cortisone preparations are also used. Cortisone is anti-inflammatory and slows down the overreaction of the immune system. Usually, cortisone is given for a week to slowly reduce the dosage within one to two weeks (“tapering” of cortisone therapy).

Treatment of a severe Purpura Schönlein-Enoch

When kidney involvement occurs, the so-called creatinine level in 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 the creatinine level is slightly elevated for more than six weeks (less than two grams of creatinine per gram of urine: 2 g / g) is used, high-dose cortisone preparations are possible, which are given over a period of about twelve weeks, with the dosage being gradually reduced again in the last few weeks (“tapering off”).

Renal involvement may cause increased blood pressure. In this case, the doctor will medically set your child to normal blood pressure. He will also recommend that you have your child’s kidney function checked regularly for up to two years after the birth of Schönlein-Henoch nephritis.

Purplura Schönlein-Enoch: Disease course and prognosis

A purine Schönlein-Enoch usually heals by itself after a few days. In severe cases, skin and soft tissue necrosis (dying tissue parts) can form, which spontaneously heal with scarring. The healing process can then last between four and six weeks. In very rare cases, it comes Purpura Schönlein-Enoch to terminal kidney failure.