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. Therapy for children with henochschonlein purpura nephritis. Though short term results in hsp nephritis have been good, long term prognosis in those with major. The aim of this study was to estimate the association of hsp with drug and vaccine administration in a pediatric population. Differences between adult and pediatric onset henochschonlein purpura from north india. It is generally a benign, selflimited disorder that follows an intercurrent illness, usually of the upperrespiratory tract.
We present a case of neuroblastoma in a 7yearold boy diagnosed after suspected hsp. It is managed in the outpatient setting and rarely associated with malignancy. Henoch schonlein purpura hsp, iga vasculitis what is henoch schonlein purpura hsp, iga vasculitis. Key points about henoch schonlein purpura hsp henoch schonlein purpura hsp is a condition that involves swelling inflammation of small blood vessels. The annual incidence of primary vasculitis in children and adolescents younger than 17 years old is approximately 23 per 100,000. To study the clinical characteristics of hsp in children. Henochschoenlein purpura american academy of pediatrics. Purpura not due to a low platelet count, caused by inflammation in blood vessels of the skin, is the hallmark of hsp. Henochschonlein purpura archives of disease in childhood. Henochschonlein purpura hsp pediatric research and. Nov 16, 2018 please use one of the following formats to cite this article in your essay, paper or report. Henochschonlein purpura is the most common systemic vasculitis of childhood.
Pdf analysis of seasonal tendencies in pediatric henoch. Gastrointestinal manifestations have been long recognized as part of the disease, but usually preceded by. Atypical purpura location in a pediatric patient with. However, all four elements of this tetrad are not required for diagnosis. Our case highlights the importance of maintaining a broad differential diagnosis in children with atypical hsp and performing a skin biopsy with. Frequently, hsp leads to abdominal pain and intestinal bleeding, renal involvement and joint pain. Iga vasculitishenoch schonlein purpura igavhsp is the most common childhood vasculitis in most parts of the world. Henochschonlein purpura is a self limiting disease characterised by a tetrad of clinical manifestations that vary in occurrence and order of presentation. Henochschonlein purpura american academy of pediatrics. Henoch schonlein purpura hsp is a small vessel vasculitis that annually affects 10 to 20 children per 100,000 population. 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.
Jul 24, 2019 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. Pdf iga vasculitis henochschonlein purpura in children. 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. Hallmarks of henochschonlein purpura include a characteristic rash, migratory polyarthritis, renal involvement, and gi involvement. In the skin, the disease causes palpable purpura small, raised areas of bleeding underneath the skin, often with joint pain and abdominal pain. Henoch schonlein purpura hsp is the most common vasculitis syndrome of childhood. Urinalysis is the only investigation required in a classic presentation of hsp. Henochschonlein purpura hsp is the most common form of vasculitis in children. The microscopic hallmark of hsp is the deposition of iga an. Should children with henoch schonlein purpura and abdominal pain be treated with steroids. The vasculitic nature of hsp, or anaphylactoid purpura, was. 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. Henoch schonlein purpura is the most common form of vasculitis in pediatric age group.
The diagnosis is made clinically and 95% of patients will present with a rash, together with any from a triad. Usually, the immune system makes antibodies, or proteins, to protect the body from foreign substances such as bacteria or viruses. Henochschonlein purpura hsp, spring fever pediatrics course. Henoch schonlein purpura hsp is a wellcharacterized immune complexmediated small vessel vasculitis of childhood with an incidence of 6. Henochschonlein purpura hsp is a common systemic vasculitis affecting children. Henochschonlein purpura hsp is the most common vasculitis in children, affecting 8 to 20 children per 100 000 annually. Henochschonlein purpura rheumatology medbullets step 23. Histopathology and correlates of systemic disease in adult henochschonlein purpura.
A diagnosis of pancreatitis complicating henochschonlein purpura was made. Henochschonlein purpura is caused by an abnormal immune system response in which the bodys immune system attacks the bodys own cells and organs. Henochschonlein purpura hsp is the most common systemic vasculitis in children 1. Henochschonlein purpura in children pubmed central pmc. Henoch schonlein purpura also known as iga vasculitis, is often called hsp for short. Henochschonlein purpura can involve nearly any organ system. Henochschonlein purpura in children, adults pictures. Henochschonlein purpura in a pediatric patient with lupus article pdf available in archives of medical science 3. Other symptoms include painful swelling around the joints and abdominal pain. Petechiae and purpura home department of pediatrics. Henochschonlein purpura is a medical condition in which there is inflammation and bleeding in the small blood vessels of the skin, joints, intestines, and kidneys.
It is the most frequent vasculitis in children and the incidence in adults varies. Apr 16, 2018 henochschonlein purpura hsp is the most common systemic vasculitis in children. Concomitantly, the serum amylase level became elevated and leukocytosis developed, with a shift to the left. Hsp is an autoimmune disease that is often triggered by an upper. The swollen blood vessels leak into the skin, joints, intestines, and kidneys.
This smallvessel vasculitis is mediated by immunoglobulin a igacontaining immune complexes and is characterized by nonthrombocytopenic purpura, abdominal pain, arthralgias, and renal disease. The classic feature of henochschonlein purpura in children and adults is a purple colored rash which forms usually in the lower legs and the hips. Nov 08, 2019 henochschonlein purpura hsp is a rare disease that causes the blood vessels in your skin to become irritated and swollen. Pdf henochschonlein purpura in a pediatric patient with lupus. The classic symptom of hsp is a red to dark purple rash, called purpura, which is often most severe on the legs and buttocks. 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. The peak incidence of pediatric hsp occurs during the autumn and winter. Henoch schonlein purpura hsp is a disease involving inflammation of small blood vessels. Pdf henochschonlein purpura hsp is the most common vasculitis of. A 5yearold girl suffering from henochschonlein purpura developed severe abdominal pain accompanied by vomiting and fever.
It is seen most often in children between the ages of 2 and 6. Aug 16, 2012 schonleinhenoch syndrome is a form of systemic smallvessel vasculitis,characterised by vascular andor mesangial immunoglobulin a1 deposits. Henochschonlein purpura nephritis in children world journal of. Typically seen in the legs and buttocks but it can seen in other parts of the body as well like arms, trunk, and face. The child may also have systemic symptoms including abdominal pain, polyarthralgia and signs of renal. It can occur in any age and peaks around 46 years old. The first clinical description of henochschonlein purpura hsp comes. The inflammation causes blood vessels in the skin, intestines. Pediatric henochschonlein purpura childrens national. Pancreatitis as a complication of henochschonlein purpura. Henochschonlein purpura hsp is a condition that involves swelling inflammation of small blood vessels.
Jul 26, 2017 henochschonlein purpura hsp is a disease that causes small blood vessels to become inflamed and leak blood. It is also known as anaphylactoid purpura, leukocytoclastic vasculitis, allergic vasculitis, and rarely, as rheumatoid purpura. It is the most common form of systemic vasculitis in. Henochschonlein purpura hsp is an igamediated systemic small vessel vasculitis. Henochschonlein purpura in children trnka 20 journal of. The diagnosis is made clinically and 95% of patients will present with a rash, together with any from a. Henoch schonlein purpura or hsp is a common childhood vasculitis, most commonly occurring in children ages 3 to 15 years of age.
Henochschonlein purpura is an iga vasculitis characterized by a tetrad of palpable purpura. Henoch schonlein purpura hsp is a systemic vasculitic disease which is. Iga is a protein in your body that usually protects your body from infections. Although hsp is seen in infancy through adulthood, most documented cases affect children. Henoch schonlein purpura hsp is a type of vasculitis, which means inflammation of the blood vessels. The rash is usually below the waist in the legs and buttocks.
Most hsp symptoms, such as temporarily palpable purpura, gastrointestinal gi. 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. It demonstrates seasonal variation implicating a role for environmental triggers and geographical variation. It gets its name from two german doctors, johann schonlein and eduard henoch, who. Schonleinhenoch purpura hsp is a systemic vasculitis that affects vessels of a small calibre. Learn more about henochschonlein purpura hsp research and clinical trials from experts at boston childrens, ranked best childrens hospital by us news.
Question a child recently presented to my office with lower limb petechiae, arthralgia, and abdominal pain characteristic of henochschonlein purpura hsp. Generally, the prognosis of hsp in children is favorable, but there are severe complications, one of which is renal involvement. Genetic research may reveal the potential role of cytokines, endothelia and nitric oxide metabolism in henochschonlein purpura. The most striking feature of this form of vasculitis is a purplish rash, typically on the lower legs and buttocks. There is no single diagnostic test to confirm henoch schonlein purpura. Pulmonary, cardiac, genital and neurological symptoms have also be observed. In addition, there may be varying degree of renal involvement. Vasculitis means inflammation of tiny blood vessels. When the blood vessels bleed, you get a rash called purpura. Henochschonlein purpura hsp is the most common vasculitis in childhood.
Henoch schonlein purpura is an idiopathic, iga associated, systemic smallvessel vasculitis characterized by the clinical tetrad of palpable purpura, arthralgias, renal dysfunction, and abdominal. Childhood iga vasculitis henoch schonlein purpuraadvances. Hsp can affect blood vessels in the bowel and the kidneys, as well. Chen o1,2, zhu xb1, ren p5, wang yb3, sun rp1, wei de4. Henochschonlein purpura is an idiopathic, iga associated, systemic smallvessel vasculitis characterized by the clinical tetrad of palpable purpura, arthralgias, renal dysfunction, and abdominal. The main clinical manifestations are vascular purpura, predominating on the lower limbs, and articular, gastrointestinal and renal symptoms. Henochschonlein purpura and drug and vaccine use in. Pedscases podcast scripts pediatric education online. The tetrad of purpura, arthritis, kidney inflammation, and abdominal pain is often observed. Introduction cassio lynm, ma in jama, february 15, 2012 vol 307, no. Epidemiological studies have shown hsp to have an annual incidence of approximately. In the majority of children, the outcome of henochschonlein purpura is excellent with spontaneous resolution of symptoms and signs. It is a multisystem disease most commonly affecting skin, joints, gastrointestinal tract, and kidneys, but other organs may be affected. Risk of long term renal impairment and duration of follow up recommended for henochschonlein purpura with normal or minimal urinary.1023 827 1313 1414 1285 453 408 680 1215 864 1229 1254 1242 945 1159 1080 473 1274 72 879 411 141 574 691 534 1397 13 154 278 1336 827 1387 438