Henochschonlein purpura also known as anaphylactoid purpura is a type of vasculitis that affects small blood vessels and characterized by elevated immunoglobulin a iga. Animal models of hsp are needed to better understand the mechanism of hsp. Henochschonlein purpura with hypocomplementemia in children. Headache is rather common in this small vessel vasculitis 1, 2. Henoch schonlein purpura is a condition that causes inflamed, leaky blood vessels. Feb 04, 2020 henochschonlein purpura hsp is a vasculitis that most commonly occurs in children.
The disease is characterized by the tetrad of palpable purpura, arthritisarthralgia, abdominal pain, and renal manifestations. An acute respiratory infection precedes purpura in a high proportion of affected young children. Relapses occur in about one third of patients, typically within 4 months from the initial presentation and with milder symptoms. It frequently occurs following an infectious trigger and involves iga and c3 deposition in small vessel walls. Clinical course of extrarenal symptoms in henoch schonlein purpura.
Henoch schonlein purpura hsp is an immunemediated vasculitis. The result is inflammation in the microscopic blood vessels in. Although it can occur at any age, hsp is overwhelmingly a disease of childhood. The lesions are usually 2 to 10 mm in diameter, and represent the extravasation of blood into the skin. Pathologically, it can be considered a form of immune complexmediated leukocytoclastic vasculitis lcv involving the skin and other organs. Azathioprine therapy for steroidresistant henochschonlein. Hsp is characterised by the classic tetrad of rash, abdominal pain, arthritisarthralgia, and glomerulonephritis.
Hsp presents with purple spots on the skin purpura, arthralgia, digestive problems, and kidney injury. In a minority of patients, the kidneys become involved and nephritis develops. Hsp is the most common vasculitis in children, with an incidence of about 10 cases per 100 000 a year. Jun 23, 2016 henochschonlein purpura hsp is the most common form of systemic vasculitis in children. Palpable purpura on the lower extremities of a child from the collection of paul f. Jul 26, 2017 henochschonlein purpura hsp is a disease that causes small blood vessels to become inflamed and leak blood. Serum ape is known to be associated with thrombotic events. Henochschonlein purpura with gastrointestinal involvement in. Henochschonlein purpura anaphylactoid purpura is the most common connectivetissue disorder in children. It is a multisystem disease most commonly affecting skin, joints, gastrointestinal tract, and kidneys, but other organs may be affected. There are no available figures for this condition, but it is not an especially common disorder. Henochschonlein purpura is a small vessel vacuities in which complexes of immunoglobulin a iga and complement component 3 c3 are deposited on arterioles, capillaries, and venules. The clinical implications of adultonset henochschonelin. The rash of hsp usually is accompanied by arthralgia, abdominal pain, and renal disease.
Anesthetic management of a patient with henochschonlein. A proposed pathway for followup watson l 1,2, richardson a 1, holt r. Pdf henochschonlein purpura with intussusception in an adult. Iga vasculitis is a disease that involves purple spots on the skin, joint pain, gastrointestinal problems, and glomerulonephritis a type of kidney disorder. These files will have pdf in brackets along with the filesize of the download. 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. It is characterized by a purpuric rash, painful swollen joints, and abdominal pain with vomiting. Meagher, jr denver, colorado this report describes the successful use of abdominal ultrasound to diagnose intussusception in a patient with henochschonlein purpura. 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 with gastrointestinal involvement. She received her medical degree from new york medical college in valhalla, new york. Figure caption and citation for the preceding image starts.
It tends to occur in the pediatric population peak incidence 310 years 3. Create a free personal account to download free article pdfs, sign up for alerts, customize your interests, and more. 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. Nevertheless, when it does occur the signs and symptoms are sufficiently severe to cause alarm and at times difficulties in diagnosis.
Successful treatment of hemorrhagic bullous henochschonlein. Iga vasculitis is caused by an abnormal response of the immune system. The most striking feature of this form of vasculitis is a purplish rash, typically on the lower legs and buttocks. Numerous palpable purplish plaques on the bilateral legs. Henochschonlein purpura if you would like to discuss your kidney diagnosis with our trained members of staff ring the free to call number 0800 169 0936. The presence of nonthrombocytopenic palpable purpura is essential to the diagnosis and the purpura are mostly located on the dependent parts like lower extremities and buttocks. Henoch schonlein purpura clinical medicine health care. Glomerulonephritis and gastrointestinal bleeding are common complications. The dilemma of spontaneous recovery even in patients with severe clinical and histologic. Henochschonlein purpura hsp, spring fever symptoms and.
Hsp is characterized by palpable purpura plus iga deposition on biopsy, arthritisarthralgia, renal involvement hematuria andor proteinuria, andor abdominal. Children may develop arthritis inflammation of the. Henochschonlein purpura hsp is the most common vasculitis of childhood and affects the small vessels. Pocket companion to brenner and rectors the kidney eighth edition, 2011. It is characterized by a clinical triad of palpable purpura without thrombocytopenia, abdominal pain, and arthritis. Henochschonlein purpura with adalimumab therapy for. Henochschonlein purpura hsp is a multisystem disease and immunoglobulin amediated vasculitis with a selflimited course affecting the skin, joints, gastrointestinal tract, and kidneys. The disease presents characteristically as a skin rash associated with joint inflammation arthritis and cramping pain in the abdomen. Henoch schonlein purpura is an inflammation of the small blood vessels of the skin, joints, bowels and kidneys. The authors found no ape in the csf of two control individuals or in the serum of two patients with. If you do not have it you can download adobe reader free.
Arthropathy in hypersensitivity reactions classd elswhr. Although retrospective studies suggest beneficial effects of some therapies, prospective randomized clinical trials proving treatment efficacy are still lacking. A form of blood vessel inflammation that typically affects small arterial vessels capillaries in the skin, kidneys, and intestinal tract. Henochschonlein purpura archives vasculitis foundation. Palpable purpura age tufts clinical and translational science institute 9711 11. Henochschonlein purpura hsp, spring fever symptoms and diagnosis see online here henochschonlein purpura hsp, also called immunoglobulin a vasculitis igav, is an igamediated disorder and the most common systemic case of vasculitis in children. Henochschonlein purpura hsp is a small vessel vasculitis mediated by igaimmune complex deposition. Severe renal and central nervous system disease may lead to lifethreatening conditions, and immunosuppressive agents and plasmapheresis may be needed. It is characterized by the clinical tetrad of nonthrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. Kaitlin quinn, md, is the recipient of the 2017 vcrcvf fellowship. Hematologic and immunological characteristics of henoch. Papules and hemorrhagic macules resembling hives are apparent. The photo depicts the foot of a child affected by the condition. Henochschonlein purpura hsp is one of the most common vasculitides of childhood, with 1020 cases per 100,000 children.
Skin lesions are characterized as palpable purpura and are typically nonblanching. Because of its systemic nature, involvement of other organ systems such as cardiopulmonary, genitourinary, and nervous system may be observed 25. F 24, left leg click on the figs for enlarged view diagnosis. Based on the hypothesis that an inherited predilection to hypercoagulability may predispose to hsp or may mark those who develop acute clinical manifestations, we evaluated the possible roles of methylenetetrahydrofolate reductase mthfr gene c677t, factor v fv gene g1691a leiden, and. In the skin, the disease causes palpable purpura small, raised areas of bleeding underneath the skin, often with joint pain and abdominal pain. Hsp is the most common form of vasculitis in childhood and affects about 20 in 100,000 children. The patient had experienced multiple hospitalizations in the prior 12 months and had demonstrated chronic kidney disease, with a serum creatinine level in the 2. Problems of classification of henoch schonlein purpura.
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. Iga immunoglobulin a vasculitis causes inflammation and bleeding of the small blood vessels of the skin, joints. Henochschonlein purpura hsp is a smallsized vasculitis affecting mainly children. Recent findings this article will summarize recent work in molecular biology and genetics evaluating predisposing. 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. Henoch schonlein purpura nephritis is a rare kidney disease leading to chronic kidney disease in a nonnegligible percentage of patients. Henoch schonlein purpura is a systemic smallvessel vasculitis that typically affects the skin of the lower extremities and can also affect the gastrointestinal tract and kidneys.
It predominantly affects the skin, joints, gastrointestinal. It gets its name from two german doctors, johann schonlein and eduard henoch, who. As with iga nephropathy, serum levels of iga are high in hsp and there are identical findings on renal biopsy. Henochschonlein purpura symptoms and causes mayo clinic. Henochschonlein purpura hsp is a systemic smallvessel immunoglobulin ig adominant vasculitis characterized by palpable purpura, hematuria, abdominal pain, and arthritis. Henochschonlein purpura with ileitis terminalis springerlink. Iga vasculitis formerly known as henoch schonlein purpura hsp is a type of nonthrombocytopenic immunemediated small vessel acute leukocytoclastic vasculitis. Here we present a case of an adult male with ulcerative colitis who developed hsp while being treated with. Journal of pediatric gastroenterology and nutrition. There is some evidence to support steroid therapy in the treatment of severe abdominal pain, severe nephritis, and central nervous system involvement. We report here on a 24yearold man with gastrointestinal pain in whom the classical features of henochschonlein purpura appeared only 6 days after acute abdominal symptoms. Nov 25, 2015 background henochschonlein purpura hsp or anaphylactoid purpura. Gastrointestinal manifestations are observed in 5075% of patients. A case of henochschonlein purpura with dilated coronary.
The estimated annual incidence of hsp in china is 14. Henochschonlein purpura associated with celiac disease. It is the most common vasculitis of childhood, and typically presents in children aged between 28. However, the routine use of corticosteroids is controversial. To open a pdf file you will need compatible software such as adobe reader. Longterm outcome studies are still defining how this disease affects a small but significant percentage of patients afflicted with henochschonlein purpura. Although hsp is seen in infancy through adulthood, most documented cases affect children. Henoch schonlein purpura hsp is a disease that causes small blood vessels to become inflamed and leak blood.
Hsp can affect blood vessels in the bowel and the kidneys, as well. Henochschonlein purpura hsp is an acute vasculitic syndrome presenting with cutaneous purpura, arthritis, and gastrointestinal and renal impairment generally seen in children. Gastrointestinal manifestations of henochschonlein purpura commonly include abdominal pain and gastrointestinal bleeding as well as extraintestinal signs and symptoms. With kidney involvement, there may be a loss of small amounts of blood and. Trapani s, micheli a, grisolia f, resti m, chiappini e, falcini f, et al. A 65yearold man presented with a 1week history of abdominal pain and was noted to have acute kidney failure, with a serum creatinine level of 3. More than 90 percent of patients are children younger than 10 years, with a peak incidence at six years of. It is the most common acute systemic vasculitis in childhood and mainly affects skin, gastrointestinal tract, joints, and kidneys. Henochschonlein purpura with intussusception in an adult article pdf available in the american journal of the medical sciences 3444. Epidemiological studies have shown hsp to have an annual incidence of approximately.
Henochschonlein purpura childrens hospital of philadelphia. Additionally, the patient reported a bilateral burning sensation from the knees to the feet. It occurs most commonly in children ages 26, although it can occur at any age. Pdf most of the documents on the racgp website are in portable document format pdf. Jan 17, 2015 henoch schonlein purpura refers to an acute or chronic vasculitis affecting primarily small vessels of the skin, joints, g. Henochschonlein purpura national kidney federation. A diagnosis of henochschonlein purpura is fairly easy to make if the. The clinical course and prognosis of henochschonlein purpura hsp associated with hypocomplementemia are not clear. 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. A case of henochschonlein purpura hs p gwyneth evans. Henochschonlein purpura hsp is an immunemediated vasculitis. Henochschonlein purpura american academy of pediatrics. When the blood vessels bleed, you get a rash called purpura. The skin is a major target organ, and the hallmark of hsp is palpable cutaneous purpura.
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