Palpable purpura age may 27, 2011 henoch schonlein purpura hsp is a small vessel vasculitis mediated by igaimmune complex deposition. Alfredo cs1, nunes na, len ca, barbosa cm, terreri mt, hilario mo. 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. Henochschonlein purpura hsp is the most frequent vasculitis in. Thrombocytopenia palpable purpura does not occur in thrombocytopenia. Matthias beck phd fachbereichsprufung kranial osteopathie 05. In a minority of patients, the kidneys become involved and nephritis develops. Henochschonlein purpura hsp, also known as anaphylactoid purpura, purpura rheumatica, and schonleinhenoch purpura is a disease of the skin and other organs that most commonly affects children.
Henochschonlein purpura, leucocitoclastic vasculitis. Therapy for children with henochschonlein purpura nephritis. Articles from medical journal, armed forces india are provided here courtesy of elsevier. Causes of palpable purpura rocky mountain spotted fever acute meningococcemia disseminated gonococcal infection ecthyma gangrenosum henoch. Up to one third of patients may develop pain, erythema, and swelling of the scrotum and spermatic cord, most commonly in boys younger than 7 years of age. Meetings take place in a variety of locations including the welsh. Henochschonlein purpura hsp is an acute immunoglobulin a iga. This small vessel vasculitis is characterised by palpable purpura, abdominal pain, arthritis or arthralgia and kidney involvement.
It is characterized by the clinical tetrad of nonthrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. Endoscopy of the small bowel shows ulcerous hemorrhagic enteritis, together with a wide range of elementary lesions. Iga vasculitis is caused by an abnormal response of the immune system. Find publications indiana university school of medicine. Henoch schonlein purpura hsp is an immune mechanism leukocytoclastic vasculitis with small vessel involvement. It affects children, mainly males, in 90% of cases the four main clinical features include purpura rash, abdominal pain, arthralgia and renal involvement. Mit unseren standardwerken pflege heute, mensch korper krankheit. Multiple bacterial and viral infectious agents have been associated with the development of henochschonlein purpura, and cases of henochschonlein purpura also have been reported after drug ingestions and vaccinations. Hspn is caused by the glomerular deposition of immunoglobulin a1 iga1containing immune complexes in the mesangium, the. The topic of the paper is henoch schonlein purpura. The characteristic pathological feature of this vasculitis is iga immune complex deposition in vascular walls of the affected organs. Highdose methylprednisolone pulse therapy for treatment of. Pathologically, it can be considered a form of immune complexmediated leukocytoclastic vasculitis lcv involving the skin and other organs.
Iga vasculitis is a disease that involves purple spots on the skin, joint pain, gastrointestinal problems, and glomerulonephritis a type of kidney disorder. Apr 15, 2014 henochschonlein purpura hsp is the one of most common types of systemic vasculitis in childhood. Feb 2017 news update henoch schonlein purpura support. Pdf henochschonlein syndrome and selective iga deficiency. Neurologic manifestations of schonlein henoch purpura. There are no available figures for this condition, but it is not an especially common disorder. This article is part of an expert video encyclopedia. Henoch schonlein purpura support together we are stronger. Its national rare disease day, all around the uk and other countries people raise awareness. Medicationsgiven in emergency situation, if applicable, and maintenance meds 6. Henochschonlein purpura is an immunoglobulin amediated, small vascular inflammatory disease that can be associated with palpable purpura, arthralgia, abdominal pain, or nephritis. Short description of disease patho, etiology, congenital or acquired 2. Despite many years trying to get ourselves noticed we are still yet to become a registered charity. Essays in private law in memory of professor david carey miller.
Medicine mcq 149 causes of palpable purpura pg blazer. 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. Palpable purpura could occur in the following conditions, except. It is characterized by a purpuric rash, painful swollen joints, and abdominal pain with vomiting. Severe hemorrhagic bullous lesions in henoch schonlein purpura. Henoch schonlein purpura hsp is the commonest systemic vasculitis of childhood typically presenting with a palpable purpuric rash and frequently involving the renal system. Role of the 70kda subunit of human replication protein a i. The result is inflammation in the microscopic blood vessels in. Firstly we are still relatively small and entirely voluntary run. Adult onset immunoglobulin a vasculitis henochschonlein purpura.
Henochschoenlein purpura is a leukocytoclastic vasculitis of childhood characterized by abdominal pain, bloody diarrhea, petechiae, and potential risk of renal involvement. Henochschonlein purpura symptoms and causes mayo clinic. Singlestranded dna binding activity, but not polymerase stimulatory activity, is required for dna replication. Since it was first described in the 19th century, research has demonstrated that the classic clinical signs and symptoms of hsp are due to an immunoglobulin a igamediated smallvessel leukocytoclastic vasculitis. Spalding, paul rosen, in comprehensive pediatric hospital medicine, 2007. Henochschonlein purpura anaphylactoid purpura is the most common connectivetissue disorder in children. Immunoglobulin a vasculitis igav, formerly called henochschonlein purpura hsp 1, is the most common form of systemic vasculitis in. Interventions for preventing and treating kidney disease. Nevertheless, when it does occur the signs and symptoms are sufficiently severe to cause alarm and at times difficulties in diagnosis. We are the first group to clinically assess, critically analyse and subsequently revise a nurse led monitoring pathway for this condition. Treatment of hsp, indications for different medications. This disease is mainly characterized by palpable purpura, arthritis arthralgia. We report on a 35yearold woman who developed palpable purpura with necrotizing cutaneous lesions on the lower limbs. Schonleinhenoch purpura, anaphylactoid purpura rhumatoid purpura diagnosis diagnosis of hsp is established when at least 2 of the following 4 criteria are present.
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