Penyakit ini ditandai oleh lesi kulit spesifik berupa purpura nontrombositopenik, artritis, nyeri perut dan perdarahan saluran cerna, serta dapat pula disertai nefritis. As with the other forms of vasculitis, the cause of hsp is not known. Henoch schonlein purpura, nephritis, therapy, children introduction henoch schonlein purpura hsp is an immunologically mediated disease associating purpura plus urticaria, characteristically affecting lower limbs, buttocks, and elbows. Medical files of patients with hsp admitted at the department of pediatrics, plovdiv, were studied retrospectively for a fiveyear. Also known as allergic purpura, anaphylactoid purpura, and schonleinhenoch syndrome. It usually happens in the skin, intestines, and kidneys. When the blood vessels bleed, you get a rash called purpura. Henochschonlein purpura hsp for parents nemours kidshealth. Henochschonlein purpura also known as anaphylactoid purpura is a type of vasculitis that affects small blood vessels and characterized by elevated immunoglobulin a iga. Symptoms include a purple spotted skin rash, abdominal pain and gastrointestinal upsets such as diarrhoea. Jul 26, 2017 henochschonlein purpura hsp is a disease that causes small blood vessels to become inflamed and leak blood. The iga deposits in vessel walls, associated with the.
Longterm outcome and treatment academic dissertation to be presented with the assent of the faculty of medicine, university of oulu, for public discussion in the auditorium 12 of the department of paediatrics, on november 25th, 2005, at 12 noon oulun yliopisto, oulu 2005. In the skin, the disease causes palpable purpura small, raised areas of bleeding. Henoch schonlein purpura causes skin rash, pain in the abdomen, and joint inflammation. Henochschonlein purpura and drug and vaccine use in. Henoch schonlein purpura also known as iga vasculitis is a disorder that causes the small blood vessels in your skin, joints, intestines, and kidneys to become inflamed and bleed.
Henochschonlein purpura is an iga vasculitis characterized by a tetrad of palpable purpura. Henoch schonlein purpura is a small vessel vasculitis that affects mainly pediatric patients, characterized by the tetrad of palpable purpura, abdominal pain, arthralgia or arthritis. The main clinical features are skin purpura, arthritis, abdominal pain, gastrointestinal bleeding, and nephritis. Jun 18, 2016 henochschonlein purpura hsp is the most common vasculitis in childhood. The disease is characterized by abnormal deposits of immunoglobulin a an antibody in the blood vessels, leading to their inflammation vasculitis. It is the most common form of childhood vascular inflammation vasculitis and results in inflammatory changes in the small blood vessels. Hsp results from inflammation of the small blood vessels in the skin and various other tissues within the body. The diagnosis and classification of henochschonlein. The inflammation causes blood vessels in the skin, intestines, kidneys, and joints to start leaking. Henoch schonlein purpura also known as anaphylactoid purpura is a type of vasculitis that affects small blood vessels and characterized by elevated immunoglobulin a iga. Probably, an external stimulus such as an infectious agent provokes an immunopathological reaction in a genetically predisposed host, with leukocytoclastic vasculitis and deposition of iga immune complexes.
This smallvessel vasculitis is mediated by immunoglobulin a igacontaining immune complexes and is characterized by nonthrombocytopenic purpura, abdominal pain, arthralgias, and renal disease. The rash is purpuric due to bleeding into the skin and there be hives and areas of redness. Henoch schonlein purpura is an inflammation of the small blood vessels of the skin, joints, bowels and kidneys. The tetrad of purpura, arthritis, kidney inflammation, and abdominal pain is often observed. Hsp is a small vessel vasculitis the major manifestations of which include. Henoch schonlein purpura hsp is a small vessel vasculitis mediated by igaimmune complex deposition. Henochschonlein purpura hsp affects the blood vessels and causes a spotty rash. Henoch schonlein purpura hsp is a disease involving inflammation of small blood vessels. She has torpid evolution, and gastrointestinal and renal complications. Henoch schonlein purpura hsp is an igamediated, autoimmune hypersensitivity vasculitis of childhood. Schonleinhenoch purpura shp is now classified as a form of systemic vasculitis with igadominant immune deposits affecting small vessels and typically involving skin, gut, and glomeruli and associated with arthralgias or arthritis, although only the skin is involved in 100% of cases. Henoch schonlein purpura hsp, also called immunoglobulin a vasculitis igav, is a vascular disease that primarily affects small blood vessels.
Background 1st described in 1801 by william heberden, a physician in london, who wrote about a case of a 5 year old boy with hematuria, abdominal pain, joint pains and skin rash. Henochschonlein purpura hsp, also called immunoglobulin a vasculitis igav, is a vascular disease that primarily affects small blood vessels. However, all four elements of this tetrad are not required for diagnosis. It is characterized by the clinical tetrad of nonthrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. Each of the forms of vasculitis tends to involve certain characteristic blood vessels. Palpable purpura age henoch schonlein purpura hsp is a shortterm inflammation of certain blood vessels vasculitis thought to be triggered by a malfunction of the immune system. The inflammation causes blood vessels in the skin, intestines. Purpura henoch schonlein phs atau disebut juga sebagai purpura anafilaktoid adalah sindrom klinis yang disebabkan oleh vaskulitis pembuluh darah kecil sistemik. Symptoms of this disease include skin lesions, joint pain, blood in urine hematuria, and abdominal pain. Dec 11, 2018 schonleins name should come first because he was the first person to recognize the condition and did so in 1837. Central nervous system cns involvement in henochschonlein purpura hsp is rare but poses diagnostic difficulties.
Health professionals diagnosis henochschonlein purpura through blood tests elevated iga. The aim of this study was to estimate the association of hsp with drug and vaccine administration in a pediatric population. There are many different conditions that feature vasculitis. Hsp may be associated with an upper respiratory tract infection or possibly an allergic reaction. Inflamed blood vessels in the skin can leak blood cells, causing a. Henochschonlein purpura hsp is a form of leukocytoclastic or smallvessel vasculitis, most often observed in children. Clinical course of extrarenal symptoms in henoch schonlein purpura. The treatment of henoch schonlein purpura is directed toward the most significant area of involvement. The small vessels of the skin, joints, kidneys, and digestive organs are particularly involved. Inflamed blood vessels in the skin can leak blood cells, causing a rash called purpura. This page includes the following topics and synonyms. Henochschonlein purpura hsp is the most common vasculitis in children, affecting 8 to 20 children per 100 000 annually. Henochschonlein purpura is the most common systemic vasculitis of childhood.
Children particularly boys are most commonly affected, although the. Nov 25, 2015 background henochschonlein purpura hsp or anaphylactoid purpura. Symptoms can begin in children, most commonly between the ages of 4 and 7 years, soon after an upper respiratory tract infection or a streptococcalpharyngitis sore throat infection. The clinical implications of adultonset henoch schonelin. Henoch schonlein purpura was confirmed by finding iga deposition on vessels within gastric and duodenal mucosa, while immunohistochemistry also identified dense focal t cell infiltration in. Henoch schonlein purpura, who presented to our department with cutaneous manifestations. Henochschonlein purpura hsp is an immunologically mediated disease associating purpura plus urticaria, characteristically affecting lower limbs, buttocks, and elbows.
Henochschonlein purpura in children pubmed central pmc. The most striking feature of this form of vasculitis is a purplish rash, typically on the lower legs and buttocks. The disease presents characteristically as a skin rash associated with joint inflammation and cramping pain in the abdomen. Henoch schonlein purpura triggered by bee sting is a rare picture. Henochschonlein purpura nord national organization for. A case of hsp is reported in a 36yearold female with. Central nervous system involvement in henochschonlein.
Hsp affects the small vessels called capillaries in. The main symptom of hsp is a rash of raised red or purple spots. Welcome to the magic world of nephropathology, glomeruli, podocites, interstituim and so on in kidney pathology. When blood vessels get inflamed, they can bleed into the skin, causing a reddishpurple rash purpura. Henochschonlein purpura hsp is the most common form of vasculitis in children. Henochschonlein purpura symptoms and causes mayo clinic. Health professionals diagnosis henoch schonlein purpura through blood tests elevated iga. Henochschonlein purpura hsp is a form of blood vessel inflammation or vasculitis. Henochschonlein purpura hsp is a rare inflammatory disease of the small blood vessels capillaries and is usually a selflimited disease. Dec 10, 2018 henoch schonlein purpura hsp, also called immunoglobulin a vasculitis igav, is a vascular disease that primarily affects small blood vessels.
Clinical course of extrarenal symptoms in henochschonlein purpura. Henochschonlein purpura american academy of pediatrics. It is sometimes called anaphylactoid purpura hsp results from inflammation of the small blood vessels in the skin and various other tissues within the body hsp generally affects children, but it may also occur in adult life. Purpura not due to a low platelet count, caused by inflammation in blood vessels of the skin, is the hallmark of hsp. Multiple bacterial and viral infectious agents have been associated with the development of henochschonlein purpura, and cases of henochschonlein purpura also have been reported after drug ingestions and vaccinations. We use your linkedin profile and activity data to personalize ads and to show you more relevant ads. Henoch in 1868 reported the first case of a patient with colic, bloody diarrhea, painful joints, and a rash. Henoch added descriptions of gastrointestinal gi involvement in 1874 and renal involvement in 1899. Schonleinhenoch purpura, anaphylactoid purpura rhumatoid purpura diagnosis diagnosis of hsp is established when at least 2 of the following 4 criteria are present.
Henochschonlein purpura, henoch schonlein purpura, henochschoenlein purpura, henoch schoenlein purpura, hsp, iga vasculitis, immunoglobulin a. Its not usually serious, but can sometimes lead to kidney problems. It gets its name from two german doctors, johann schonlein and eduard henoch, who. Henochschonlein purpura hsp, the commonest childhood vasculitis, is characterised by nonthrombocytopaenic palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement. A form of blood vessel inflammation that typically affects small arterial vessels capillaries in the skin, kidneys, and intestinal tract. The peak incidence is between the ages of 4 years and 8 years with a male preponderance. Introduction cassio lynm, ma in jama, february 15, 2012 vol 307, no. Iga immunoglobulin a vasculitis causes inflammation and bleeding of the small blood vessels of the skin, joints. Some include gastrointestinal hemorrhage as a fourth criterion. Hsp can affect blood vessels in the bowel and the kidneys, as well. Children may develop arthritis inflammation of the. The aim of this study was to estimate the association of hsp with drug and vaccine administration in a pediatric. Henochschonlein purpura hehnok shoonline purrpyuhruh is a condition that makes small blood vessels get swollen and irritated. Jun 20, 2019 henochschonlein purpura hsp is an igamediated, autoimmune hypersensitivity vasculitis of childhood.
Disease modifying antirheumatic agents dmards or biologics have been successfully used to treat those refractory cases. It is important to conduct a good medical history in all patients in which henoch schonlein purpura is suspected, with information that includes, exposure to drugs, infectious diseases, and insect bites to decipher the potential trigger. Henochschonlein purpura hsp is by far the most frequently occurring type of vasculitis in childhood. Frequently, hsp leads to abdominal pain and intestinal bleeding, renal involvement and joint pain. Azathioprine is a dmard that has been reported to be effective in hsp nephritis and in adult. Purpura, arthritis, and abdominal pain are known as the classic triad of henochschonlein purpura. The prognosis for patients with henoch schonlein purpura is generally excellent. Therapy for children with henochschonlein purpura nephritis. In the majority of children, the outcome of henochschonlein purpura is excellent with spontaneous resolution of symptoms and signs.
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