Henoch-Schonlein purpura, also known as HSP, is a type of vasculitis, a group of disorders that cause blood vessel inflammation. In HSP, the inflammation causes bleeding in the capillaries in the skin, joints, intestines and kidneys. Although HSP can affect anyone, it occurs most often in children between the ages of 2 and 11, affecting boys more often than girls. HSP is the most common form of vasculitis in children, with an annual occurrence of 140 cases per one million children. However, adult cases are sometimes more difficult. Skin manifestations are more variable in adults, and sometimes symptoms in adults endure longer.
The cause of the initial inflammation is still unknown, but is believed that the immune …show more content…
systems play a role in targeting the blood vessels involved. A possible factor in some cases is an abnormal response to certain triggers by the immune system which is generally why HSP is considered an autoimmune disorder. Some of the triggers include viral and bacterial infections such as strep throat and parvovirus infection. HSP is also linked to certain vaccinations, including those for typhoid, cholera, yellow fever, measles, or hepatitis B, genetically modified foods, certain medicines, including some types of antibiotics and antihistamines, and certain chemicals. Some experts also say that HSP is associated with the colder weather of fall and winter. Another common trend of HSP is that it develops days after symptoms of an upper respiratory tract infection have developed. The most distinctive characteristic of HSP is the reddish-purple rash, which resemble the appearance of a bruise. An indication of this type of rash is that is does not blanch. The rash typically develops on the lower legs and buttocks, but is not unusual to appear on the arms, head, trunk and areas of pressure such as the sock line or waist line. People with HSP often have painful, swollen joints primarily in the knees and ankles. The joint pain usually precedes the rash by a day or two. These symptoms subside once the disease is gone and leaves no lasting damage. Another symptom associated with HSP is the development of gastrointestinal complications, including abdominal pain, nausea, vomiting and bloody stool. These types of symptoms tend to develop eight days after the classical rash first appears. In rare cases, an abnormal folding of the bowel (intussusception) may cause a bowel blockage, which may require surgery to fix. HSP can also affect the kidneys which are indicated by signs such as blood or protein in the urine. These signs are usually found through urine tests, due to it generally not causing discomfort. Usually this goes away once the illness passes, but in a few cases, kidney disease may develop and even persist into kidney failure. Adults with HSP are more likely to develop extended kidney problems, while children are more likely to develop complications including severe abdominal pain and gastrointestinal bleeding. In some cases, Henoch-Schonlein purpura causes serious problems of the bowel or kidneys, so it’s important to see a doctor as soon as the rash or other symptoms develop. A diagnosis is fairly easy with the three main symptoms are present, but when only one or two of the symptoms are present certain tests can help rule out other diseases and make a diagnosis of HSP seem likely. One of these tests includes a blood test which is used to reveal an elevated level of protein called IgA which suggests Henoch-Schonlein purpura, but it isn 't conclusive. Another test is a urine test used to determine kidney function which reveals if the kidneys have been indeed affected. If it has been confirmed that the kidneys have been affected a kidney biopsy may be needed to determine the severity. Imaging studies may also be necessary to rule out other causes for the abdominal pain such as appendicitis. This disease usually improves on its own within a few weeks with no long lasting ill effects, but bouts of the rash may recur.
The treatment is usually aimed at preventing further complications, most often meaning bed rest, plenty of fluids, and acetaminophen (Tylenol, others) or nonsteroidal anti-inflammatory drugs, such as ibuprofen (Advil, Motrin, others), costing on average $8 per bottle . If the kidneys are affected, medical care is generally needed, as well as long-term follow-up to prevent more-serious problems. Depending upon the severity of the kidney damage the treatments can range from steroidal drugs, such as prednisone, costing $100 per bottle without insurance, to a kidney transplant, costing $262,900 without …show more content…
insurance. HSP is named after Eduard Heinrich Henoch, a German pediatrician, and his teacher Johann Lukas Schönlein who described this disease during the 1860s. Schönlein linked the purpura (rash) with arthritis and Henoch linked purpura with gastrointestinal involvement. Although HSP had been named after Henoch and Schönlein, the disease was already described relatively in 1802 by an English physician William Heberden and dermatologist Robert Willian. The first name of Heberden-Willian disease had fallen in disuse. The first to identify the underlying allergic mechanism was William Osler, a founding professor at John Hopkins Hospital. HSP does not usually cause any impairment in people’s lives.
People with HSP are encouraged to stay comfortable and allow for the disease to run its course. Urine tests do need to be done periodically which can be quite frustrating especially for one who a busy schedule. Since HSP does not typically affect people’s lifestyle in a major way there is only a small amount of research currently being done. The main research being done is studies to fully understand the underlying causes so they can stop the disease from developing into severe problems such as renal kidney failure. They are doing so by many clinical trials using different medications to determine which ones essentially treat the disease which will in return show what underlying factor is causing the
disease. I personally chose this disease to research because I have this disease and I honestly knew very little about it besides the fact that it gave me this horrific looking rash and caused me to go to the hospital at least once a week for some sort of diagnostic test including blood tests, urinalysis’, and even a kidney biopsy. The main sources of my report are WebMD and my mother, who, working in the medical field and being with me through all appointments and procedures, helped me better understand the disease. I thought learning the unusual triggers of this disease was the most interesting. I do wish I knew what exactly causes the disease because it is still unknown and experts have simply suggested that those unusual triggers have been linked to HSP. I feel my report demonstrates a personal understanding of the disease but I wish I had more of a personal approach, rather than just cold, hard facts, considering I do have this disease.
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Bibliography:
"Henoch-Schonlein Purpura (HSP): Causes, Symptoms, and Treatment." WebMD. WebMD, n.d.
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"Henoch-Schonlein Purpura: MedlinePlus Medical Encyclopedia." U.S National Library of
Medicine. U.S. National Library of Medicine, n.d. Web. 15 May 2013.
Staff, Mayo Clinic. "Definition." Mayo Clinic. Mayo Foundation for Medical Education and
Research, 04 Nov. 2010. Web. 15 May 2013.
Urinary and Digestive system