If a child or baby develops a fever with swelling of the extremities again after a flu-like infection or childhood illness has already resolved, Purpura Schoenlein-Henoch should be considered. This condition is also associated with pinpoint skin hemorrhages that sometimes look like blood blisters.
What is purpura Schoenlein-Henoch disease?
Purpura Schoenlein-Henoch is an inflammatory disease of the blood vessels triggered by the immune system. Initially, mild fever as well as pain may occur; later, the small and medium-sized vessels in particular lose stability, and blood may penetrate the overlying skin. This results in the punctiform skin bleeding typical of this disease, also known as petechiae. In addition, there are swellings on the backs of the feet and hands due to the retention of tissue fluid in these areas. Affected children are conspicuous by a sudden restriction of movement. In some cases, Purpura Schoenlein-Henoch can also cause inflammation of the kidneys or intestines, as the disease can also manifest itself on the mucous membranes. Bloody diarrhea and blood in the urine then occur. The disease progresses in episodes lasting several weeks. Affected are mainly children up to school age, but hardly babies or adolescents.
Causes
Purpura Schoenlein-Henoch is in most cases a consequence of a previous illness such as chickenpox, rubella, or measles and occurs up to three weeks after these infections. Schoenlein-Henoch purpura can also occur after a simple flu-like infection or after an infection with influenza A viruses and other diseases that primarily affect the upper respiratory tract. The intake of medication is very rarely causative for this immune reaction. In individual cases, therefore, there is no identifiable cause for the occurrence of the disease. It is not yet known why Purpura Schoenlein-Henoch manifests itself. However, allergic reactions of the patient to the pathogens are suspected as the trigger for the sudden inflammatory reactions.
Symptoms, complaints, and signs
The first symptoms usually begin suddenly. They do not show up continuously, but come in episodes. Children feel ill, suffer from loss of appetite, abdominal cramps, and headaches. As the disease progresses, the characteristic signs of Purpura Schoenlein-Henoch follow: small punctate hemorrhages (petechiae) initially only a few millimeters in diameter. Over time, they enlarge and join together to form larger spots. They are most commonly found on the buttocks and lower legs. They do not itch, cannot be pushed away, are slightly raised and palpable. As a rule, they are distributed symmetrically on the body. Furthermore, petechiae can also appear in the digestive tract, causing bloody diarrhea and colicky abdominal cramps. They are also possible on the kidneys, causing bloody urine. When they affect the joints, there is restricted movement and swelling, often in the knee and ankle joints. In boys they form very rarely sometimes on the testicles or arms. In exceptional cases, the brain can be affected by the petechiae, leading to paralysis, seizures or even impaired consciousness. An extreme complication of the disease is when the spots begin to bleed and become necrotic, so that the tissue dies. In this case, the child’s life is in danger.
Diagnosis and course
Purpura Schoenlein-Henoch is diagnosed by the physician on the basis of the typical circular and merging skin hemorrhages. These occur on the lower abdomen and buttocks, as well as on the lower legs and feet. In boys, they may additionally be visible on the testicles. Other symptoms such as abdominal pain and bloody diarrhea indicate bowel involvement. However, these symptoms do not appear until about one to two weeks after the skin reactions. Dysfunction of the kidneys is also possible at a later stage. Then blood and protein are found in the urine during laboratory tests. In addition, blood pressure may also be elevated. More than 30 percent of children with the disease suffer from kidney dysfunction in the course of Purpura Schoenlein-Henoch.
Complications
Purpura Schoenlein-Henoch leads to a number of different unpleasant symptoms in the patient. Usually, it results in hemorrhage on the skin.These can occur in various parts of the body and are not infrequently accompanied by pain. Likewise, the affected regions of the body are not infrequently swollen and there is pain in the joints. Due to the joint pain, patients not infrequently also suffer from restricted movement and thus difficulties in everyday life. Pain in the abdomen or stomach can also occur due to Schoenlein-Henoch purpura. Furthermore, blood also appears in the stool and urine. The blood pressure itself is also increased by Purpura Schoenlein-Henoch, so that other diseases can occur more easily. As a rule, no special treatment of this disease is necessary. With the help of bed rest and relaxation, the body can fight the disease. Likewise, medications can continue to support the treatment. Complications usually do not occur. The patient’s life expectancy is also not affected if the disease is successfully treated. Without treatment, Purpura Schoenlein-Henoch can also lead to renal failure.
When should you see a doctor?
Medical treatment is always necessary for Schoenlein-Henoch purpura. There is no self-healing in this disease, and the disease usually cannot be treated by self-help means. Only medical treatment can prevent further complications and discomfort. A doctor should be consulted if the affected person suffers from severe pain in the abdomen and loss of appetite. There may also be pain in the head or small hemorrhages all over the body. If these complaints occur permanently and do not disappear on their own, a doctor must be consulted in any case. Purpura Schoenlein-Henoch must also be treated in case of severe diarrhea. Furthermore, swelling or restrictions in movement can also indicate the disease and should be examined by a doctor. The sooner Schoenlein-Henoch purpura is treated, the greater the likelihood of a complete recovery. In the worst case, the tissue may die completely.
Treatment and therapy
In most cases, Purpura Schoenlein-Henoch heals on its own in a few days. There are no medications specifically effective against this condition. The child should be on bed rest to protect him or her. In cases of bloody diarrhea, the immune response is weakened with cortisone so that the intestines can recover. Treatment is more difficult if the kidney function progressively deteriorates. In the case of so-called Schoenlein-Henoch nephritis, the severity of the disease must be determined by a kidney biopsy. Kidney function can be improved by administering cortisone or other drugs that artificially suppress the immune system. Increased blood pressure resulting from the kidney disease is also treated with appropriate medications. In this severe course of Purpura Schoenlein-Henoch, it takes about four to six weeks for the child to fully recover. After the disease, the kidney function must be checked at regular intervals for several years, as it may deteriorate again in the course of the disease. However, the occurrence of kidney failure or shock due to sudden onset of extensive skin hemorrhages is extremely rare. Purpura Schoenlein-Henoch may occur repeatedly in children.
Prevention
There are no measures to prevent purpura Schoenlein-Henoch. If unusual skin symptoms are discovered in babies and younger children, a doctor should be consulted as soon as possible. This will ensure monitoring in case of a possible severe course of Purpura Schoenlein-Henoch.
Follow-up
Purpura Schoenlein-Henoch does not require specific causal follow-up. Because it is a multisystem disease, various complaints may exist that are treated symptomatically. In severe cases of the disease, cortisone therapy may be prescribed. Prescribing immunosuppressants may also be necessary. In addition, the use of analgesics may be advised for pain and persistent itching. Supportive antipruritic ointments may also be used. After the disease, affected persons should take it easy, if necessary keep to bed rest and avoid intensive physical activities. Fish oil capsules can have a positive effect on healing. Tight clothing should also be avoided so that the skin can also recover.In case of impaired kidney function or kidney failure caused by advanced Schoenlein-Henoch purpura, kidney transplantation or lifelong dialysis is necessary. Since there is an increased tendency of relapses in this disease, regular check-ups are necessary for up to two years. In particular, the kidney function is checked with the help of regular urine samples. The prognosis for Schoenlein-Henoch purpura, especially in children, tends to be positive. Healing occurs within one to several months. Subsequent quality of life is generally not affected in mild to normal cases. The exception is when renal damage has occurred.
