Henochschonlein purpura hsp is vasculitis of the small vessels, the most common vasculitis of the childhood and is uncommon in adults. The vast majority of people with henochschonlein purpura hsp recover on their own within several weeks. Henoch schonlein purpura, henoch schonlein purpura, henoch schoenlein purpura, henoch schoenlein purpura, hsp, iga vasculitis, immunoglobulin a vasculitis. The main symptom of hsp is a rash of raised red or purple spots.
It may be the result of the immune system responding inappropriately to certain triggers. Henochschonlein purpura hsp is a rare inflammatory disease of the small blood vessels capillaries and is usually a selflimited disease. Clinical course of extrarenal symptoms in henochschonlein purpura. Henochschonlein purpura hsp is a form of leukocytoclastic or smallvessel vasculitis, most often observed in children. In describing hsp, heberden wrote of a 5yearold boy who was seized with pains and swellings in various parts.
Henochschonlein purpura and drug and vaccine use in childhood. The disease is characterized by abnormal deposits of immunoglobulin a an antibody in the blood vessels, leading to their inflammation vasculitis. E o mais comum na infancia e e causado por um fenomeno autoimune. When blood vessels get inflamed, they can bleed into the skin, causing a reddishpurple rash purpura. The underlying pathogenesis is uncertain however the structural end organ injury is caused by the deposition of iga molecules due to a reduction in glycosylation. Palpable purpura age 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. Its not clear why this initial inflammation develops. Each of the forms of vasculitis tends to involve certain characteristic blood vessels.
Henoch schonlein purpura, sometimes known as hsp, is an autoimmune condition that causes inflammation in the tiny blood vessels of the skin. Kdigo 2012 clinical practice guideline for the evaluation and management. Henoch schonlein purpura causes inflammation of small blood vessels, which then become easily broken and allow blood to leak. We are the first group to clinically assess, critically analyse and subsequently revise a nurse led monitoring pathway for this condition. Dental assisting training video for dental office staff. Henochschonlein purpura definition of henochschonlein.
Renal manifestations of henochschonlein purpura in a 6month prospective study of 223 children. Also known as allergic purpura, anaphylactoid purpura, and schonleinhenoch syndrome. The prognosis for patients with henoch schonlein purpura is generally excellent. Henochschonlein purpura is a self limiting disease characterised by a tetrad of clinical manifestations that vary in occurrence and order of presentation. Henoch schonlein purpura nephritis hspn and igan are considered nowadays as related diseases, since both have been described in identical twins 7 and bear identical pathological and biological abnormalities. In henochschonlein purpura, some of the bodys small blood vessels become inflamed, which can cause bleeding in the skin, abdomen and kidneys.
Henochschoenlein purpura is a leukocytoclastic vasculitis of childhood characterized by abdominal pain, bloody diarrhea, petechiae, and potential risk of renal involvement. It is not clear what triggers the condition, but it often appears a few weeks after an upper respiratory infection such as strep or mycoplasma. Purpura, arthritis, and abdominal pain are known as the classic triad of henochschonlein purpura. A small percentage of children with henochschonlein purpura hsp develop a chronic form of the disease that often requires prolonged corticosteroid therapy. Henoch schonlein purpura hsp guideline gl604 approval approval group job title, chair of committee date paediatric governance policy and procedure subcommittee. Hsp results from inflammation of the small blood vessels in the skin and various other tissues within the body. Henochschonlein purpura hsp is a vasculitis inflammation of blood vessels that affects small blood vessels mainly in the skin, intestines, and kidneys. Schonleinhenoch purpura hsp is a systemic vasculitis that affects vessels of a small calibre. 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.
Dec 11, 2018 schonleins name should come first because he was the first person to recognize the condition and did so in 1837. A case of hsp is reported in a 36yearold female with. Henochschonlein purpura allergy and clinical immunology. Iga is a form of antibody that we all make, to protect the lining of the airway, throat, and gut. Please use one of the following formats to cite this article in your essay, paper or report. Endoscopy of the small bowel shows ulcerous hemorrhagic enteritis, together with a wide range of elementary lesions. Dr ann gordon, chair paediatric governance september 2012 change history version date author, job title reason 1 09072012 kate hunter spr new guideline contents. Henoch schonlein purpura hsp or iga vasculitis, is the most common childhood vasculitis. The inflammation causes blood vessels in the skin, intestines, kidneys, and joints to start leaking. Henochschonlein purpura circulatory system and disease. Eventually these spots blend to form bigger areas of bruising purpura in the skin. In the skin, the disease causes palpable purpura small, raised areas of bleeding. The treatment of henoch schonlein purpura is directed toward the most significant area of involvement.
Jul 26, 2017 henochschonlein purpura hsp is a disease that causes small blood vessels to become inflamed and leak blood. As with iga nephropathy, serum levels of iga are high in hsp and there are identical findings on renal biopsy. It gets its name from two german doctors, johann schonlein and eduard henoch, who. The inflammation causes blood vessels in the skin, intestines. Children may develop arthritis inflammation of the. Iga vasculitis is a disease that involves purple spots on the skin, joint pain, gastrointestinal problems, and glomerulonephritis a type of kidney disorder. Henoch schonlein purpura hsp royal berkshire hospital. Hypersensitivity vasculitis and henoch schonlein purpura. Introduction to hsp incidence, aetiology, diagnostic criteria. Henochschonlein purpura anaphylactoid purpura, vascular purpura internal medicine an acquired form of small vessel vasculitis with igadominant immune deposits affecting small vesselsarteries, capillaries, venules, typically of the skin, gut, and glomeruli, associated with arthralgia andor arthritis. Henoch schonlein purpura hsp, the commonest childhood vasculitis, is characterised by nonthrombocytopaenic palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement.
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. Endoscopy revealed diffuse ulcerations in the stomach, duodenum, and right colon. Hsp is most common in younger pts clinical red maculopapules on legs and buttocks. The aim of the present study on the occurrence of henochschonlein purpura hsp in dutch children is to give some insight into the epidemiology of hsp in the netherlands, to record the diagnostic criteria used by dutch paediatricians and to evaluate the accuracy of the latter using the presence of iga in the skin when biopsies are available. Henochschonlein purpura hsp is the most common vasculitis in childhood. Schonleinhenoch purpura, anaphylactoid purpura rhumatoid purpura diagnosis diagnosis of hsp is established when at least 2 of the following 4 criteria are present. Biopsies revealed a leukocytoclastic vasculitis in the skin and gastrointestinal tract. The rash is purpuric due to bleeding into the skin and there be hives and areas of redness. Children younger than 2 years with hsp are more likely to develop edema swelling of various areas of their bodies, which is a result of leaky small blood vessels in the skin. Henoch schonlein purpura hsp is an immunologically mediated disease associating purpura plus urticaria, characteristically affecting lower limbs, buttocks, and elbows. 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. Frequently, hsp leads to abdominal pain and intestinal bleeding, renal involvement and joint pain.
It is the most common form of childhood vascular inflammation vasculitis and results in inflammatory changes in the small blood vessels. Should children with henoch schonlein purpura and abdominal pain be treated with steroids. Henochschonlein purpura symptoms and causes mayo clinic. Henochschonlein purpura nord national organization for. This guideline has been adapted for statewide use with the support of the victorian paediatric clinical network. There is no single diagnostic test to confirm henoch schonlein purpura. Henoch schonlein purpura causes skin rash, pain in the abdomen, and joint inflammation. The henochschonlein purpura hsp origa vasculitis is the most.
The estimated annual incidence of hsp in china is 14. Disease modifying antirheumatic agents dmards or biologics have been successfully used to treat those refractory cases. Henochschonlein purpura also known as anaphylactoid purpura is a type of vasculitis that affects small blood vessels and characterized by elevated immunoglobulin a. A case report of a child with purpura, severe abdominal. 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. Henoch schonlein purpura causes a purple spotted skin rash which lasts around one to four weeks, and is often marked by relapses. Hsp affects the small vessels called capillaries in. Nov 25, 2015 background henochschonlein purpura hsp or anaphylactoid purpura. A diagnosis of henochschonlein purpura was rendered. Henoch schonlein history henoch schonlein purpura support. Apr 30, 2014 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.
This page includes the following topics and synonyms. Steroidswere notadministered because shebegan a progressiverecoveryon day15, and was discharged on day 34 with resolution of the rash, joint pain, headache, and. Most children and many adults with acute leukaemia can expect to be cured, while chronic leukaemia can be successfully managed. The disease presents characteristically as a skin rash associated with joint inflammation and cramping pain in the abdomen. The profile and clinical outcomes of patients with renal. Symptoms can begin in children, most commonly between the ages of 4 and 7 years, soon after an upper respiratory tract infection or a. Symptoms include a distinctive rash and swelling in the joints. The vast majority of people with henochschonlein purpura hsp recover on. Dec 10, 2018 henoch schonlein purpura hsp, also called immunoglobulin a vasculitis igav, is a vascular disease that primarily affects small blood vessels. A form of blood vessel inflammation that typically affects small arterial vessels capillaries in the skin, kidneys, and intestinal tract. We describe a case of an adolescent male with henoch schonlein purpura hsp, presenting with cutaneous and gastrointestinal manifestations.
Henoch schonlein purpura is an inflammation of the small blood vessels of the skin, joints, bowels and kidneys. There are many different conditions that feature vasculitis. Henoch in 1868 reported the first case of a patient with colic, bloody diarrhea, painful joints, and a rash. Its not usually serious, but can sometimes lead to kidney problems. Patient information bookthe patient information book includes general information on vasculitis, the individual diseases, medications, tips for managing the disease and other resources. A nurse led hsp renal monitoring care pathway has been. Click here to download the patient information book. Children may also develop abdominal pain that can be quite severe. Seek immediate medical attention after the onset of hsp symptoms. Henochschonlein purpura hsp is the most common vasculitis in children, affecting 8 to 20 children per 100 000 annually. The iga deposits in vessel walls, associated with the. Answer henochschonlein purpura is a common and selflimiting disease in. This article is part of an expert video encyclopedia. Some include gastrointestinal hemorrhage as a fourth criterion.
Henoch schonlein purpura hsp is the commonest systemic vasculitis of childhood typically presenting with a palpable purpuric rash and frequently involving the renal system. The history of henoch schonlein started with dr william heberden, a london physician, described the first cases of henoch schonlein hee. Aug 23, 2014 37 purpura henoch schonlein kriteria ara definisi purpura non trombositopenik palpable purpura lesi kulit hemoragik yang dapat diraba, terdapat elevasi kulit, tidak berhubungan dengan trombositopenia usia onset hsp affects the blood vessels and causes a spotty rash. Steroid therapy led to complete resolution of the symptoms.