Criteria for identifying henochschoniein pur pura hsp and distinguishing hsp from other forms of systemic arteritis were developed by comparing the manifestations in 85 patients who had hsp with those of 722 control patients with other forms of vasculitis. In patients with hsp, the persons own immune system attacks blood vessels in the skin, intestines, joints and. Henochschonlein purpura also known as iga vasculitis, is often called hsp for short. Generalised vasculitis of small vessels aetiology unclear. Henochschonlein purpura, nephritis, therapy, children introduction henochschonlein purpura hsp is an immunologically mediated disease associating purpura plus urticaria, characteristically affecting lower limbs, buttocks, and elbows. The inflammation causes blood vessels in the skin, intestines.
Frequently, hsp leads to abdominal pain and intestinal bleeding, renal involvement and joint pain. Henochschonlein 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 hsp does affect children more frequently than adults. Organ involvement in immunoglobulin a vasculitis henoch. Pdf henochschonlein purpura hsp is the most common vasculitis of childhood.
Henochschonlein purpura archives vasculitis foundation. Henochschonlein purpura hsp is an autoimmune disorder that is due to the production of autoantibodies against some of the bodys own antigens. Epidemiological studies have shown hsp to have an annual incidence of approximately. Henochschonlein purpura is the most common childhood vasculitis, but may also affect adults. By the traditional format of choosing different combinations. Henochschonlein vasculitis hsv is systemic small vessel vasculitis involving the skin, kidney, joints, and gastrointestinal tract.
Schonlein, henoch, and later osler and others elucidated a broad spectrum of signs and symptoms that were associated with purpura and smallvessel vasculitis, including arthritis, peripheral neuropathy, abdominal pain. The most striking feature of this form of vasculitis is a purplish rash, typically on the lower legs and buttocks. Henoch schonlein purpura anaphylactoid purpura, hsp ncbi. Ig a vasculitis is the most common nonthrombocytopenic purpura in childhood. Henochschonlein purpura causes skin rash, pain in the abdomen, and joint inflammation. Jul 14, 2016 henochschonlein purpura hsp is generally a selflimited vasculitis disease and has a good prognosis. Henochschonlein purpura hsp is a shortterm inflammation of certain blood vessels vasculitis thought to be triggered by a malfunction of the immune system. Iga vasculitis formerly known as henoch schonlein purpura is a form of blood vessel swelling, also known as vasculitis.
Apr 17, 20 the vast majority of people with henoch schonlein purpura hsp recover on their own within several weeks. 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. We report a 4yearold thai boy who presented with palpable purpura, abdominal colicky pain, seizure, and eventually developed intestinal ischemia. It is not one, but a multitude of diseases with a wide variety of manifestations, ranging from skin involvement alone to widespread lifethreatening damage to multiple organs.
Hsp occurs more frequently in africans than caucasians b. Henochschonlein purpura is a particular form of blood vessel inflammation called vasculitis. Henoch schonlein purpura hsp is a type of vasculitis, which means inflammation of the blood vessels. Medical files of patients with hsp admitted at the department of pediatrics, plovdiv, were studied retrospectively for a fiveyear. Symptoms of this disease include skin lesions, joint pain, blood in urine hematuria, and abdominal pain. A case report with sequential magnetic resonance imaging.
The swelling is due to an abnormal response of the immune system. The clinical implications of adultonset henochschonelin. The disease is a clinical syndrome characterized by multiple organ involvement including the skin, joints, gastrointestinal tract, and the kidneys. Henoch schonlein purpura or hsp is a common childhood vasculitis, most commonly occurring in children ages 3 to 15 years of age. Usually, the immune system makes antibodies, or proteins, to protect the body from foreign substances such as bacteria or viruses. This disease involves vasculitis of the small blood vessels swelling of these vessel walls around the body, and leads to effects across a variety of organs. Schonleinhenoch purpura shp is a systemic iga vasculitis that affects small vessels. In most affected individuals, the condition goes away on its own. Henochschonlein purpura hsp is an immunoglobulina iga mediated small vessel vasculitis that predominantly affects children but is also seen in.
Henoch schonlein purpura can affect anyone, but its most common in children between the ages of 2 and 6. Gastrointestinal manifestations of henoch schonlein purpura. Henochschonlein purpura hsp, also called immunoglobulin a vasculitis igav, is a vascular disease that primarily affects small blood vessels. Henoch schonlein purpura causes skin rash, pain in the abdomen, and joint inflammation. Pathologically, it can be considered a form of leukocytoclastic vasculitis that can involve not only the skin but other tissues as well.
However, hsp develops into a chronic condition in up to 5% of individuals, which means they continue to have symptoms into adulthood. Apr 16, 2018 henochschonlein purpura hsp is the most common systemic vasculitis in children. The proportion of patients reported to have renal involvement varies between 20% and 80%. Henochschonlein purpura or hsp is a common childhood vasculitis, most commonly occurring in children ages 3 to 15 years of age. Although diagnosis in the primary care setting may be difficult, it is vital in order to avoid significant complications. This is due to the immune system product called iga immunoglobulin. Henochschonlein purpura hsp, iga vasculitis what is henochschonlein purpura hsp, iga vasculitis. The documents contained in this web site are presented for information purposes only. Iga vasculitis, formerly henochschonlein purpura, is a form of vasculitisa family of rare disorders characterized by inflammation of the blood vessels, which can restrict blood flow and damage vital organs and tissues. Case report gastrointestinal mucosal lesions in an adult with. In the skin, the disease causes palpable purpura small, raised areas of bleeding. Supportive care until recovery when there is little or no kidney involvement may include adequate hydration, rest, and overthecounter pain medication as needed for joint and abdominal pain due to inflammation.
It is multisystemic small vessel vasculitis, 50%80% of which typically involves the. The rash is usually below the waist in the legs and buttocks. Apr 20, 2020 henochschonlein purpura hsp is a nonthrombocytopenic small vessel vasculitis that typically runs a benign, selflimited course and is characterized by a purpuric skin eruption usually confined. Henoch schonlein purpura can also cause abdominal pain and aching joints. Rapidly progressive glomerulonephritis in a child with. Henochschonlein purpura hsp is a systemic, generalized vasculitis of unknown origin that primarily affects children but can also occur in adult patients.
Necrosis of vessel walls resulting in aneursymal dilatation andor thrombosis causing organ ischemia, infarction, or hemorrhage. Henochschonlein purpura symptoms and causes mayo clinic. Henochschonlein purpura frequently follows an infection of the throat or breathing passages, but it can be induced by certain medications. Stenosis andor occlusion of involved vasculature resulting in organ ischemia or infarction. Dec 10, 2018 henoch schonlein purpura hsp, also called immunoglobulin a vasculitis igav, is a vascular disease that primarily affects small blood vessels. Children particularly boys are most commonly affected, although the. We report a 4yearold thai boy who presented with palpable purpura, abdominal colicky pain, seizure, and eventually developed intestinal ischemia and perforation despite adequate treatment, including corticosteroid and intravenous immunoglobulin therapy. Henoch schonlein purpura hsp is a shortterm inflammation of certain blood vessels vasculitis thought to be triggered by a malfunction of the immune system. It is characterized by the clinical tetrad of nonthrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. Henochschonlein purpura with intracerebral haemorrhage.
It is characterized by skin purpura, arthritis, and abdominal andor renal involvement. Henochschonlein purpura is a vasculitis involving the small vessels of the joints. Henochscholein purpura hsp is the most common systemic vasculitis of childhood, affecting the small blood vessels. Ninety percent of cases occur in the pediatric age group. Henochschonlein purpura hsp, also known as iga vasculitis, is the most common form of systemic vasculitis in children. Vasculitis, simply put, is blood vessel inflammation. Iga vasculitis is a disease that involves purple spots on the skin, joint pain, gastrointestinal problems, and glomerulonephritis a type of kidney disorder. In contrast to many other forms of systemic vasculitis, igav hsp is selflimited in the great majority of cases. Henochschonlein purpura can also involve the central nervous system. Henochschonlein purpura hsp paediatric systemic vasculitis. It is a multisystem disease most commonly affecting skin, joints, gastrointestinal tract, and kidneys, but other organs may be affected. The disease is characterized by abnormal deposits of immunoglobulin a an antibody in the blood vessels, leading to their inflammation vasculitis. Henochschonlein purpura archives of disease in childhood. Iga vasculitis, formerly henochschonlein purpura hsp is the most.
Henochschonlein purpura hsp is the most common vasculitic disease of childhood. Iga immunoglobulin a vasculitis causes inflammation and bleeding of the small blood vessels of the skin, joints. Iga vasculitis formerly henochschonlein purpura or hsp. The result is inflammation in the microscopic blood vessels in. Basic laboratory tests are mostly used to help ascertain the extent of organs affected and their degree vasculitis is most prevalent in patients with immunoglobulin a vasculitis henochschonlein purpura. Schonlein, henoch, and later osler and others elucidated a broad spectrum of signs and symptoms that were associated with purpura and smallvessel vasculitis, including arthritis, peripheral neuropathy, abdominal pain, pulmonary hemorrhage, epistaxis and nephritis2832. Hsp may be misdiagnosed as another form of vasculitis most commonly hypersensitivity vasculitis because of the frequent failure to perform direct immunofluorescence dif testing on skin biopsy and the consequent failure to detect iga. Henochschonlein purpura allergy and clinical immunology. There is no single diagnostic test for any of the vasculitides. Vasculitis espanol american college of rheumatology. Henochschonlein purpura from vasculitis to intestinal. Iga vasculitis is caused by an abnormal response of the immune system. Henochschonlein purpura is caused by an abnormal immune system response in which the bodys immune system attacks the bodys own cells and organs. The child may also have systemic symptoms including abdominal pain, polyarthralgia and signs of renal.
May 27, 2011 henoch schonlein purpura hsp is a small vessel vasculitis mediated by igaimmune complex deposition. Henochschonlein purpura hsp is a vasculitis inflammation of blood vessels that affects small blood vessels mainly in the skin, intestines. Incidence and risk factors for recurrent henochschonlein. It affects the small vessels called capillaries in the skin and the kidneys. 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. Symptoms include a purple spotted skin rash, abdominal pain and gastrointestinal upsets such as diarrhoea. Jul 24, 2019 the most striking feature of this form of vasculitis is a purplish rash, typically on the lower legs and buttocks.
Immunoglobulin a iga vasculitis, clinical manifestation, endoscopy, corticosteroids introduction immunoglobulin a iga vasculitis igav, formerly henochschonlein purpura, is the most common systemic vasculitis in childhood. Diagnosis henochschonlein purpura discussion henochschonlein syndrome is a hypersensitivityrelated acute smallvessel vasculitis possibly related to immunization, insect bites, medications, infections, or certain foods. Therapy for children with henochschonlein purpura nephritis. Iga vasculitis henoch schonlein purpura as the first. Henochschonlein purpura joseph junewick, md facr 010420 history 16 year old male with acute abdominal pain.
History, classification, etiology, histopathology, clinic. Gastrointestinal manifestations of henochschonlein purpura. The exact cause of hsp is unknown and there is no specific test to diagnose. Neurological complications, though rare, include focal cerebral deficit, coma, convulsion, subarachnoid hemorrhage and chorea. The small vessels of the skin, joints, kidneys, and digestive organs are particularly involved. Is called haemorrhagic vasculitis or anca negative vasculitis small vessel vasculitis involving vessels of microcirculation which are venules, capillaries and small arteries and characterised by palpable purpura. Henoch schonlein purpura frequently follows an infection of the throat or breathing passages, but it can be induced by certain medications. Henochschonlein 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 is a disease involving inflammation of small blood vessels. Henochschonlein purpura hsp is a leukocytoclastic vasculitis mediated by iga deposition that occurs mostly in children. Vasculitis means inflammation of tiny blood vessels. Central nervous system cns involvement in henochschonlein purpura hsp is rare but poses diagnostic difficulties. Small vessel vasculitis hypersensitivity vasculitis henoch schonlein purpura churg strauss syndrome wegener granulomatosis clinical manifestations of vasculitis clinical picture depends on the size and extent of the vessel involvement. Central nervous system involvement in henochschonlein.
A case of hsp is reported in a 36yearold female with. The vasculitis foundation, the worlds largest organization dedicated to providing advocacy, education and support for people with vasculitis, announces the launch of vasculitis awareness month may 2018, an annual effort to create. Henochschonlein purpura hsp is a small vessel vasculitis mediated by igaimmune complex deposition. Health professionals diagnosis henoch schonlein purpura through blood tests elevated iga. The american college of rheumatology 1990 criteria for the. Jul 26, 2017 henochschonlein purpura hsp is a disease that causes small blood vessels to become inflamed and leak blood. Cerebral vasculitis in henochschonlein purpura request pdf. Henoch schonlein purpura is a particular form of blood vessel inflammation called vasculitis.
Henochschonlein purpura hsp is vasculitis of the small vessels, the most common vasculitis of the childhood and is uncommon in adults. Immunoglobulin a iga vasculitis, clinical manifestation, endoscopy, corticosteroids introduction immunoglobulin a iga vasculitis igav, formerly henoch schonlein purpura, is the most common systemic vasculitis in childhood. It gets its name from two german doctors, johann schonlein and eduard henoch, who. Henochschonlein purpura is a disease that causes small blood vessels in the body to become inffamed and leak. Vasculitis means inflammation of the blood vessel wall. Iga is a protein in your body that usually protects your body from infections. It mostly affects children aged between 3 and 15, yet the. 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. The incidence of hsp in children is approximately 622 per 100,000 personyears 1,2,3,4, which is higher than that in adult 3. Igav is mediated by immune deposits typically with. Most hsp symptoms, such as temporarily palpable purpura, gastrointestinal gi pain, and joint pain, are selflimited. The vast majority of people with henochschonlein purpura hsp recover on their own within several weeks. Case report endoscopy and the management of iga vasculitis. Health professionals diagnosis henochschonlein purpura through blood tests elevated iga.941 96 1292 961 1332 122 667 1031 133 320 292 215 195 1132 1129 1148 10 439 1465 1217 585 991 1147 665 244 178 419 584 1489 750 825 181 950 948 474 1257 744 347 1249 910 526 681 521 1474 1159