The peak incidence is between the ages of 4 years and 8 years with a male preponderance. Hsp results from inflammation of the small blood vessels in the skin and various other tissues within the body. Henochschonlein purpura, henoch schonlein purpura, henochschoenlein purpura, henoch schoenlein purpura, hsp, iga vasculitis, immunoglobulin a. Therapy for children with henochschonlein purpura nephritis. Each of the forms of vasculitis tends to involve certain characteristic blood vessels. Henochschonlein purpura in children pubmed central pmc. This page includes the following topics and synonyms. Henochschonlein purpura hsp is a form of blood vessel inflammation or vasculitis. 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. When the blood vessels bleed, you get a rash called purpura. Henoch schonlein purpura causes skin rash, pain in the abdomen, and joint inflammation.
Hsp may be associated with an upper respiratory tract infection or possibly an allergic reaction. Henochschonlein purpura hsp affects the blood vessels and causes a spotty rash. Azathioprine is a dmard that has been reported to be effective in hsp nephritis and in adult. Henochschonlein purpura hehnok shoonline purrpyuhruh is a condition that makes small blood vessels get swollen and irritated.
The clinical implications of adultonset henoch schonelin. The inflammation causes blood vessels in the skin, intestines. A form of blood vessel inflammation that typically affects small arterial vessels capillaries in the skin, kidneys, and intestinal tract. Pathologically, it can be considered a form of leukocytoclastic vasculitis that can involve not only the skin but other tissues as well.
It is characterized by the clinical tetrad of nonthrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. Clinical course of extrarenal symptoms in henoch schonlein purpura. Purpura occur in all cases, joint pains and arthritis in 80%, and abdominal pain in 62%. Iga immunoglobulin a vasculitis causes inflammation and bleeding of the small blood vessels of the skin, joints. However, all four elements of this tetrad are not required for diagnosis. Henochschonlein purpura hsp is the most common form of vasculitis in children.
We use your linkedin profile and activity data to personalize ads and to show you more relevant ads. Henochschonlein purpura hsp is an immunologically mediated disease associating purpura plus urticaria, characteristically affecting lower limbs, buttocks, and elbows. 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. Frequently, hsp leads to abdominal pain and intestinal bleeding, renal involvement and joint pain.
The tetrad of purpura, arthritis, kidney inflammation, and abdominal pain is often observed. Central nervous system involvement in henochschonlein. Nov 25, 2015 background henochschonlein purpura hsp or anaphylactoid purpura. Purpura henoch schonlein phs atau disebut juga sebagai purpura anafilaktoid adalah sindrom klinis yang disebabkan oleh vaskulitis pembuluh darah kecil sistemik. Henoch schonlein purpura is an inflammation of the small blood vessels of the skin, joints, bowels and kidneys. 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. 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. The diagnosis and classification of henochschonlein.
Jun 18, 2016 henochschonlein purpura hsp is the most common vasculitis in childhood. Henochschonlein purpura also known as anaphylactoid purpura is a type of vasculitis that affects small blood vessels and characterized by elevated immunoglobulin a iga. The aim of this study was to estimate the association of hsp with drug and vaccine administration in a pediatric population. Henochschonlein purpura is the most common systemic vasculitis of childhood. Inflamed blood vessels in the skin can leak blood cells, causing a. Hsp affects the small vessels called capillaries in. Jun 20, 2019 henochschonlein purpura hsp is an igamediated, autoimmune hypersensitivity vasculitis of childhood. The treatment of henoch schonlein purpura is directed toward the most significant area of involvement. Henochschonlein purpura is an iga vasculitis characterized by a tetrad of palpable purpura. Introduction cassio lynm, ma in jama, february 15, 2012 vol 307, no. 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. When blood vessels get inflamed, they can bleed into the skin, causing a reddishpurple rash purpura. The prognosis for patients with henoch schonlein purpura is generally excellent. Purpura not due to a low platelet count, caused by inflammation in blood vessels of the skin, is the hallmark of hsp.
Medical files of patients with hsp admitted at the department of pediatrics, plovdiv, were studied retrospectively for a fiveyear. Henoch in 1868 reported the first case of a patient with colic, bloody diarrhea, painful joints, and a rash. The rash is purpuric due to bleeding into the skin and there be hives and areas of redness. Henochschonlein purpura hsp, the commonest childhood vasculitis, is characterised by nonthrombocytopaenic palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement. Hsp is a small vessel vasculitis the major manifestations of which include. Clinical course of extrarenal symptoms in henochschonlein purpura. 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. Henoch schonlein purpura hsp is a disease involving inflammation of small blood vessels. This smallvessel vasculitis is mediated by immunoglobulin a igacontaining immune complexes and is characterized by nonthrombocytopenic purpura, abdominal pain, arthralgias, and renal disease. Henochschonlein purpura hsp is vasculitis of the small vessels, the most common vasculitis of the childhood and is uncommon in adults. 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. Henoch schonlein purpura triggered by bee sting is a rare picture. Some include gastrointestinal hemorrhage as a fourth criterion.
A case of hsp is reported in a 36yearold female with. The inflammation causes blood vessels in the skin, intestines, kidneys, and joints to start leaking. In the skin, the disease causes palpable purpura small, raised areas of bleeding. Henochschonlein purpura hsp for parents nemours kidshealth. 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. Henochschonlein purpura hsp, also called immunoglobulin a vasculitis igav, is a vascular disease that primarily affects small blood vessels.
It gets its name from two german doctors, johann schonlein and eduard henoch, who. Hsp can affect blood vessels in the bowel and the kidneys, as well. Henochschonlein purpura american academy of pediatrics. Purpura, arthritis, and abdominal pain are known as the classic triad of henochschonlein purpura. There are many different conditions that feature vasculitis. Children particularly boys are most commonly affected, although the. Symptoms include a purple spotted skin rash, abdominal pain and gastrointestinal upsets such as diarrhoea. Henoch added descriptions of gastrointestinal gi involvement in 1874 and renal involvement in 1899.
Henoch schonlein purpura, who presented to our department with cutaneous manifestations. She has torpid evolution, and gastrointestinal and renal complications. 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. Henochschonlein purpura hsp is by far the most frequently occurring type of vasculitis in childhood. It usually happens in the skin, intestines, and kidneys. As with the other forms of vasculitis, the cause of hsp is not known. Inflamed blood vessels in the skin can leak blood cells, causing a rash called purpura. The aim of this study was to estimate the association of hsp with drug and vaccine administration in a pediatric. 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. Symptoms of this disease include skin lesions, joint pain, blood in urine hematuria, and abdominal pain.
Henochschonlein purpura hsp is a form of leukocytoclastic or smallvessel vasculitis, most often observed in children. The small vessels of the skin, joints, kidneys, and digestive organs are particularly involved. The most striking feature of this form of vasculitis is a purplish rash, typically on the lower legs and buttocks. Disease modifying antirheumatic agents dmards or biologics have been successfully used to treat those refractory cases. Henochschonlein purpura nord national organization for. Health professionals diagnosis henochschonlein purpura through blood tests elevated iga. 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. Henoch schonlein purpura hsp, also called immunoglobulin a vasculitis igav, is a vascular disease that primarily affects small blood vessels. Penyakit ini ditandai oleh lesi kulit spesifik berupa purpura nontrombositopenik, artritis, nyeri perut dan perdarahan saluran cerna, serta dapat pula disertai nefritis. Health professionals diagnosis henoch schonlein purpura through blood tests elevated iga. The main clinical features are skin purpura, arthritis, abdominal pain, gastrointestinal bleeding, and nephritis. Henochschonlein purpura symptoms and causes mayo clinic. Schonleinhenoch purpura, anaphylactoid purpura rhumatoid purpura diagnosis diagnosis of hsp is established when at least 2 of the following 4 criteria are present.
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. Dec 10, 2018 henoch schonlein purpura hsp, also called immunoglobulin a vasculitis igav, is a vascular disease that primarily affects small blood vessels. The main symptom of hsp is a rash of raised red or purple spots. Henochschonlein purpura and drug and vaccine use in. The disease presents characteristically as a skin rash associated with joint inflammation and cramping pain in the abdomen. Its not usually serious, but can sometimes lead to kidney problems. Henoch schonlein purpura hsp is an igamediated, autoimmune hypersensitivity vasculitis of childhood. The iga deposits in vessel walls, associated with the. The disease is characterized by abnormal deposits of immunoglobulin a an antibody in the blood vessels, leading to their inflammation vasculitis. In the majority of children, the outcome of henochschonlein purpura is excellent with spontaneous resolution of symptoms and signs. It is the most common form of childhood vascular inflammation vasculitis and results in inflammatory changes in the small blood vessels. 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.
Welcome to the magic world of nephropathology, glomeruli, podocites, interstituim and so on in kidney pathology. Central nervous system cns involvement in henochschonlein purpura hsp is rare but poses diagnostic difficulties. Henochschonlein purpura hsp is the most common vasculitis in children, affecting 8 to 20 children per 100 000 annually. Henochschonlein purpura hsp is a rare inflammatory disease of the small blood vessels capillaries and is usually a selflimited disease. Jul 26, 2017 henochschonlein purpura hsp is a disease that causes small blood vessels to become inflamed and leak blood. Also known as allergic purpura, anaphylactoid purpura, and schonleinhenoch syndrome. Children may develop arthritis inflammation of the. Henoch schonlein purpura hsp is a small vessel vasculitis mediated by igaimmune complex deposition.
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