Schoenlein Henoch Purpura (pronounced /ʃerˈlaɪn ˈhɛnək pɜːrˈpjʊərə/), also known as Henoch-Schönlein purpura or HSP, is a rare autoimmune disease that affects the blood vessels. The name of the disease comes from its three founders, Johann Lukas Schönlein, Eduard Heinrich Henoch, and Laurenz Gustav Adolf von Purpura. The correct spelling of each name needs to be memorized in order to properly spell the disease's name. HSP can cause joint pain, gastrointestinal problems, and a characteristic skin rash. Treatment is based on symptoms and can include steroids, NSAIDs, and immunosuppressive drugs.
Schoenlein Henoch Purpura, also known as Henoch-Schonlein purpura (HSP), is a rare childhood vasculitis disorder characterized by inflammation and small blood vessel damage, predominantly affecting the skin, joints, kidneys, and gastrointestinal tract. It is named after the physicians Johann Lucas Schönlein and Eduard Henoch, who first described the condition.
HSP is believed to occur as a result of an abnormal immune response and is often triggered by a viral or bacterial infection. It primarily affects children between the ages of 2 and 11, although it can occur in people of all ages.
One of the hallmark symptoms of HSP is purpura, which refers to the development of small, reddish-purple spots or bruises on the skin. This is accompanied by joint pain and swelling, particularly in the knees and ankles. Individuals may also experience abdominal pain, vomiting, and bloody stools if the gastrointestinal tract is affected. Additionally, HSP can lead to kidney damage, resulting in blood and protein in urine, high blood pressure, and ultimately, kidney failure in severe cases.
Diagnosis of HSP is typically based on the characteristic symptoms and a thorough physical examination. Medical tests, including blood tests, urinalysis, abdominal imaging, and kidney biopsy, may also be conducted to confirm the diagnosis and assess the severity of organ involvement.
Treatment for HSP aims to relieve symptoms and prevent complications, primarily through the use of nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce inflammation and manage pain. In severe cases, corticosteroids or other immunosuppressive medications may be prescribed. Follow-up care is essential to monitor kidney function and manage any potential long-term consequences of the disease.