Henochschonlein purpura in chinese children and adults. Pediatric nephrotic syndrome national kidney foundation. Henochschonlein purpura hsp is a vasculitis inflammation of blood vessels that affects small blood vessels mainly in the skin, intestines, and kidneys. Henoch schonlein purpura hsp most children with hsp do not require any specific treatment and recover with time alone. Petechiae and purpura home department of pediatrics. The hallmark of hsp is cutaneous palpable purpura, often involving the lower extremities and buttocks. Henoch schonlein purpura is an idiopathic, iga associated, systemic smallvessel vasculitis characterized by the clinical tetrad of palpable purpura, arthralgias, renal dysfunction, and abdominal. Igav is mediated by immune deposits typically with iga1, resulting in inflammation and necrosis of the wall of small and mediumsized blood vessels with the extravasation of erythrocytes, infiltration of. 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. Henochschonlein purpura hsp is an important cause of chronic kidney. Key points about henochschonlein purpura hsp henochschonlein purpura hsp is a condition that involves swelling inflammation of small blood vessels. Nov 08, 2019 henochschonlein purpura hsp is a rare disease that causes the blood vessels in your skin to become irritated and swollen. Henochschonlein purpura in children from western turkey.
Pedscases podcast scripts pediatric education online. The child may also have systemic symptoms including abdominal pain, polyarthralgia and signs of renal. The rash is usually below the waist in the legs and buttocks. Henochschonlein purpura refers to an acute or chronic vasculitis affecting primarily small vessels of the skin, joints, g. Henochschonlein purpura hsp is the most common vasculitic disease of childhood. Urinalysis is the only investigation required in a classic presentation of hsp. Hsp is the commonest vasculitis of childhood and is selflimiting in the majority of cases. Henochschonlein purpura hsp paediatric systemic vasculitis.
Henochschonlein purpura hsp is the most common form of vasculitis in children. While different providers will have different levels of comfort with this relatively rare diagnosis, most cases resolve on their own. Henochschonlein purpura is the most common systemic vasculitis of childhood. While different providers will have different levels of comfort with this relatively rare diagnosis, most cases resolve on. The swollen blood vessels leak into the skin, joints, intestines and kidneys. Steroid therapy led to complete resolution of the symptoms. Iga is a protein in your body that usually protects your body from infections. The following are associated with renal involvement in pediatric hsp. Therapy for children with henochschonlein purpura nephritis. Henochschonlein purpura hsp is an immunologically mediated disease associating purpura plus urticaria, characteristically affecting lower limbs, buttocks, and elbows.
Henochschonlein purpura allergy and clinical immunology. The inflammation causes blood vessels in the skin, intestines. The cutaneous manifestation of hsp is present in all patients. The syndrome of acute purpura and arthritis in children was first described by schonlein in 1837 i. The disease primarily affects young children but may affect older children and adults. Central nervous system cns involvement in henochschonlein purpura hsp is rare but poses diagnostic difficulties. When the blood vessels bleed, you get a rash called purpura. Medical files of patients with hsp admitted at the department of pediatrics, plovdiv, were studied retrospectively for a fiveyear. Gallbladder and pancreas in henochschonlein purpura.
Henochschonlein purpura hsp is a vasculitis inflammation of the blood vessels, usually involving the immune system. Introduction cassio lynm, ma in jama, february 15, 2012 vol 307, no. 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. The most common symptom is a rash, mainly on the legs, buttocks, and abdomen. Henoch schonlein purpura hsp is a wellcharacterized immune complexmediated small vessel vasculitis of childhood with an incidence of 6. Furthermore, cases of this vasculitis syndrome sometimes develop in the context of a viral hepatitis or after hepatitis vaccination methods. Jul 26, 2017 henochschonlein purpura hsp is a disease that causes small blood vessels to become inflamed and leak blood. Vasculitis from hsp henochschonlein purpura lymphoma 39. We describe a case of an adolescent male with henochschonlein purpura hsp, presenting with cutaneous and gastrointestinal manifestations.
Vasculitis means inflammation of tiny blood vessels. Henoch schonlein purpura hsp is a small vessel vasculitis that annually affects 10 to 20 children per 100,000 population. Henoch schonlein purpura in a pedi atric patient with lupus article pdf available in archives of medical science 3. Henochschonlein purpura hsp, also known as iga vasculitis, is a disease of the skin, mucous membranes, and sometimes other organs that most commonly affects children. Pdf henochschonlein purpura in a pediatric patient with. Researchers in the boston childrens kidney transplant program lead the global development of and participation in pediatric kidney transplant clinical research we are committed to developing innovative and improved antirejection medication protocols with the goal of more successful transplants and reduced side effects and complications. Basic information nephrotic syndrome ns reflects glomerular dysfunction causing proteinuria without compromising gfr occurs at all ages but is most prevalent in children between the ages 1. Original article risk factors for renal damage in henoch. Endoscopy revealed diffuse ulcerations in the stomach, duodenum, and right colon. Henoch schonlein purpura malignancy rare in children diabetes is not a cause of ns in children due to long latency. Symptoms can begin in children, most commonly between the ages of 4 and 7 years, soon after an upper respiratory tract infection or a streptococcal pharyngitis sore throat infection. In the majority of children, the outcome of henochschonlein purpura is excellent with spontaneous resolution of symptoms and signs. Biopsies revealed a leukocytoclastic vasculitis in the skin and gastrointestinal tract. Risk factors, renal damage, henoch schonlein purpura introduction henoch schonlein purpura hsp is a selflimited vasculitis occurring mostly in children 1.
Henoch schonlein purpura or hsp is a common childhood vasculitis, most commonly occurring in children ages 3 to 15 years of age. Epidemiological studies have shown hsp to have an annual incidence of approximately. In a child who presents with palpable purpura, abdominal pain, arthritisarthralgia, or renal involvement, hsp nephritis is the most likely diagnosis. This study aimed to evaluate renal involvement and factors affecting the prognosis in patients with henochschonlein purpura hsp. Hsp is primarily a disease of childhood with male predominance it often occurs after an upper respiratory infection, which is perhaps why it is often seen. In addition, there may be varying degree of renal involvement. Hsp is an autoimmune disease that is often triggered by an upper. With kidney involvement, there may be a loss of small amounts of blood and. Hsp can affect blood vessels in the bowel and the kidneys, as well. Although hsp is seen in infancy through adulthood, most documented cases affect children. Steroids given by mouth or through an iv infusion are typically given for severe abdominal pain. Histopathology and correlates of systemic disease in adult henochschonlein purpura. Original article risk factors for renal damage in henochschonlein.
Pediatric nephrotic syndrome adam goldstein howard trachtman, m. Henochschonlein purpura hsp, iga vasculitis what is henochschonlein purpura hsp, iga vasculitis. Henochschonlein purpura or hsp is a common childhood vasculitis, most commonly occurring in children ages 3 to 15 years of age. University of alberta and stollery childrens hospital in edmonton, canada, i have created this. Pdf henochschonlein purpura hsp is the most common vasculitis of. Hsp is the most common vasculitis in childhood with an incidence of 1020 cases per 100,000 in children under 17 years with a peak incidence of 70 cases per 100,000 in the 46. Usually, the immune system makes antibodies, or proteins, to protect the body from foreign substances such as bacteria or viruses. Although the term schonleinhenoch purpura is more appro priate historically, henochschoniein purpura. Gastrointestinal disorders 1 jillian sullivan, md mscs associate professor of pediatrics. Henochschonlein purpura hsp in children cedarssinai.
It gets its name from two german doctors, johann schonlein and eduard henoch, who. The estimated annual incidence of hsp in china is 14. From each report dealing with henochschonlein purpura and pancreatobiliary involvement or viral liver disease or vaccination, we excerpted data on sex, age, presence of abdominal pain preceding the purpura, aminotransferase levels, kidney disease, course, and management. Henochschonlein purpura hsp is a selflimit ed vasculitis occurring mostly in children 1. Henochschonlein purpura hsp pediatric research and. It is a multisystem disease most commonly affecting skin, joints, gastrointestinal tract, and kidneys, but other organs may be affected. Differences between adult and pediatric onset henochschonlein purpura from north india. Research open access mefv gene mutations in egyptian.
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 a systemic vasculitis that is common in the pediatric population and often presents with the classical triad of palpable purpura, arthralgia, and abdominal pain. Hsp is the most common vasculitis in childhood with an incidence of 1020 cases per 100,000 in children under 17 years with a peak incidence of 70 cases per 100,000 in the 46 year age group 1, 2. The iga deposits in vessel walls, associated with the.
The manifestations of colicky abdominal pain and of nephritis were added by henoch in 1874 2. The swollen blood vessels leak into the skin, joints, intestines, and kidneys. Should children with henoch schonlein purpura and abdominal pain be treated with steroids. Henochschonlein purpura hsp is a condition that involves swelling inflammation of small blood vessels. Children may develop arthritis inflammation of the. In the skin, the disease causes palpable purpura small, raised areas of bleeding underneath the skin, often with joint pain and abdominal pain. The american college of rheumatology 1990 criteria for the. Please use one of the following formats to cite this article in your essay, paper or report. Nov 16, 2018 please use one of the following formats to cite this article in your essay, paper or report. Pediatric henochschonlein purpura childrens national. 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. Atypical purpura location in a pediatric patient with. 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. Hsp is a multisystem disease affecting skin, joints.
Risk factors, renal damage, henochschonlein purpura introduction henochschonlein purpura hsp is a selflimited vasculitis occurring mostly in children 1. It is the most frequent vasculitis in children and the incidence in adults varies. There is no single diagnostic test to confirm henoch schonlein purpura. The aim of this study was to estimate the association of hsp with drug and vaccine administration in a pediatric. Introduction to pediatric course 473 ped dear student, welcome to the pediatric department. Henoch schonlein purpura hsp is a systemic vasculitis mediated by iga and characterised by the clinical triad of nonthrombocytopenic palpable purpura, abdominal pain and arthritis. Question a child recently presented to my office with lower limb petechiae, arthralgia, and abdominal pain characteristic of henochschonlein purpura hsp.
Apr 16, 2018 henochschonlein purpura hsp is the most common systemic vasculitis in children. Henochschonlein purpura in children from northwestern spain. An acute respiratory infection precedes purpura in a high proportion of affected young children. Hsp is a multisystem disease affecting skin, joints, gastrointestinal tract, and kidneys 2. Hsp can occur any time in life, but it is most common in children between 2 and 6 years of age. Henochschonlein purpura hsp, also called immunoglobulin a vasculitis igav, is a vascular disease that primarily affects small blood vessels. Henochschonlein purpura is an idiopathic, iga associated, systemic smallvessel vasculitis characterized by the clinical tetrad of palpable purpura, arthralgias, renal dysfunction, and abdominal. A case of henochschonlein purpura hs p gwyneth evans. Incidence and risk factors for recurrent henochschonlein. Learn more about henochschonlein purpura hsp research and clinical trials from experts at boston childrens, ranked best childrens hospital by us news. Renal damage is the most common and severe complication of hsp 3. Henochschonlein purpura hsp is the most common form of systemic vasculitis in the paediatric setting with 90% of cases occurring in childhood.
Henochschonlein purpura american academy of pediatrics. Arthritis joint pain and swelling, abdominal pain, and other symptoms can occur as well. Fifty reports published between 1977 and 2015 were retained for the analysis. Clinical features of children with henochschonlein purpura risk factors associated with renal involvement yilmaz tabel, filiz callak inanc, derya gumus dogan, ahmet taner elmas introduction. Jun 18, 2016 henochschonlein purpura hsp, recently renamed as iga vasculitis, is a systemic leukocytoplastic vasculitis characterized by iga1 dominant immune deposits 1, 2. 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 also known as iga vasculitis, is often called hsp for short. We describe a case of an adolescent male with henoch schonlein purpura hsp, presenting with cutaneous and gastrointestinal manifestations. The small vessels of the skin, joints, kidneys, and digestive organs are particularly involved. Frequently, hsp leads to abdominal pain and intestinal bleeding, renal involvement and joint pain. Henochschonlein purpura archives of disease in childhood. Should children with henochschonlein purpura and abdominal pain be treated with steroids. Henochschonlein purpura and drug and vaccine use in.
Henochschonlein purpura is an iga vasculitis characterized by a tetrad of palpable purpura. Henochschonlein purpura hsp is the most common vasculitis in children, affecting 8 to 20 children per 100 000 annually. March 2018 henochschonlein purpura hsp fibrillate into. Henoch schonlein purpura hsp is a disease involving inflammation of small blood vessels. Tags henoch schonlein purpura hsp pediatrics rick, mike, and miz discuss the differential of nonblanching rashes and the sometimesdifficult diagnosis of henoch schonlein purpura.
The disease is characterized by abnormal deposits of immunoglobulin a an antibody in the blood vessels, leading to their inflammation vasculitis. Henochschoenlein purpura american academy of pediatrics. Henochschonlein purpura is a self limiting disease characterised by a tetrad of clinical manifestations that vary in occurrence and order of presentation. Further investigations may be required if the diagnosis is unclear, abdominal symptoms are severe or where there is evidence of significant renal involvement hypertension, macroscopic haematuria or proteinuria most cases are selflimiting and require only. Central nervous system involvement in henochschonlein. The appearance of the rash in this pattern reflects its tendency to distribute in pressuredependent areas, so that in infants the rash may actually appear in upper body. Most hsp symptoms, such as temporarily palpable purpura, gastrointestinal gi pain, and joint pain, are selflimited. It is seen most often in children between the ages of 2 and 6. Clinical features of children with henochschonlein purpura. Due to an increased frequency of vasculitis in fmf patients, many investigators have studied mefv. It is seen most often in children between the ages of two and six. Dec 10, 2018 the vast majority of people with henoch schonlein purpura hsp recover on their own within several weeks. Igav is mediated by immune deposits typically with iga1, resulting in inflammation and necrosis of the wall of small and mediumsized blood vessels with the extravasation of erythrocytes, infiltration of tissue with neutrophils, and depo.
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