Henoch schonlein purpura hsp is an igamediated, autoimmune hypersensitivity vasculitis of childhood. Henochschonlein purpura in adults american journal of kidney. Henochschonlein purpura hsp is a disease involving inflammation of small blood vessels. Background 1st described in 1801 by william heberden, a physician in london, who wrote. Most children who develop hsp nephritis completely recover from the illness, whereas up to 40% of adults. The rash of hsp usually is accompanied by arthralgia, abdominal pain, and renal disease.
Henochschonlein purpura causes a purple spotted skin rash which lasts around one to four weeks, and is often marked by relapses. A presumptive diagnosis of henochschonlein purpura hsp was made based on the clinical scenario of acute kidney failure and skin findings. 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. Intravenous cyclophosphamide therapy in adults with henoch. Henoch schonlein 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 a systemic vasculitis mediated by iga and characterized by the clinical triad of nonthrombocytopenic palpable purpura. A bacteriostatic drug with antiinflammatory properties that may hasten the resolution of the palpable rash.
Henochschonlein purpura symptoms and causes mayo clinic. Henoch schonlein purpura hsp is a systemic vasculitis mediated by iga and characterized by the clinical triad of nonthrombocytopenic palpable purpura, abdominal pain, and arthritis. Pdf successful outcome of a corticodependent henoch. Henoch schonlein purpura hsp is an immunoglobulina iga mediated small vessel vasculitis that predominantly affects children but is also seen in adults 1. Henoch schonlein purpura hsp is a small vessel vasculitis mediated by igaimmune complex deposition. Schonleinhenoch purpura hsp is a systemic vasculitis that affects vessels of a small calibre. Background henoch schonlein purpura hsp, a small vessel vasculitis, is rare in the adult population. Therefore, treatment is not indicated in all cases, and full recovery is the rule. The most striking feature of this form of vasculitis is a purplish rash, typically on the lower legs and buttocks. Henochschoenlein purpura american academy of pediatrics. This guideline has been adapted for statewide use with the support of the victorian paediatric clinical network. Henoch schonlein purpura hsp frequently occurs in children under 15 years of age but is quite rare in adults.
The main clinical features are skin purpura, arthritis, abdominal pain, gastrointestinal bleeding, and nephritis. Hsp is more common in children than adults, but has a tendency to be more severe when it occurs in adults. Rest, plenty of fluids and overthecounter pain relievers may help with symptoms. Pdf henochschonlein purpura in adults researchgate. Pdf henochschonlein purpura in adults in the emergency. Henoch schonlein purpura is a condition that can cause a skin rash, tummy abdominal pain and joint pains.
It is a multisystem disease most commonly affecting skin, joints, gastrointestinal tract, and kidneys, but. Differences between adult and pediatric onset henoch schonlein purpura from north india. The inflammation causes blood vessels in the skin, intestines. Although clinical suspicion was high, the presenting complaint of shortness of breath and. Usually, the immune system makes antibodies, or proteins, to protect the body from foreign substances such as bacteria or viruses.
Henoch schonlein purpura usually goes away on its own within a month with no lasting ill effects. It is characterized by the clinical tetrad of nonthrombocytopenic palpable purpura. A cohort of 250 adults suffering hsp was retrospectively analyzed for a median followup period. All patients with henochschonlein purpura develop a purpuric rash, 75 percent develop arthritis. Henochschonlein purpura diagnosis and treatment mayo. In adults, hsp can lead to chronic kidney disease ckd and kidney failure, described as endstage renal disease when treated with bloodfiltering treatments.
The clinical implications of adultonset henochschonelin. Pdf henochschonlein purpura hsp in an adult researchgate. She has torpid evolution, and gastrointestinal and renal complications. Pdf henochschonlein purpura hsp is vasculitis of the small vessels, the most common vasculitis of the childhood and is uncommon in. Pdf a report of an unusual and easily missed diagnosis of henochschonlein purpura in an adult caucasian male who presented in the emergency. The small vessels of the skin, joints, kidneys, and digestive organs are particularly involved. The classic symptom of hsp is a red to dark purple rash, called purpura, which is often most severe. The distinctive syndrome of henoch schoenlein purpura hsp was first described by heberden before 1800, and in 1808 english physician robert willan described a patient. Henochschonlein purpura emerging treatments bmj best. We describe a case of an adolescent male with henoch schonlein purpura hsp, presenting with cutaneous and gastrointestinal manifestations. A case of hsp is reported in a 36yearold female with.
Henoch schonlein purpura hsp, also called immunoglobulin a vasculitis igav, is a vascular disease that primarily affects small blood vessels. Urinalysis is the only investigation required in a classic presentation of hsp. Longterm outcome studies are still defining how this disease affects a small but significant percentage of patients afflicted with henoch schonlein purpura. New insights in the pathogenesis of immunoglobulin a. Abstract henochschonlein purpura hsp is a small vessel vasculitis with immunoglobulin. Henochschonlein purpura in adults american journal of. It is the most frequent vasculitis in children and. Successful treatment of henochschonlein purpura nephritis. In addition, there may be varying degrees of renal involvement. The disease is characterized by abnormal deposits of immunoglobulin a an antibody in the blood vessels, leading to their inflammation vasculitis. Thus, although adult hsp is uncommon, when adults present with a purpuric rash and rpgn, besides vasculitides such as polyarteritis and thrombotic thrombocytopenic purpura, hsp should be a diagnostic consideration, particularly in the setting of possible or proven malignancy. Henoch scholein purpura hsp introduction hsp is the commonest vasculitis of childhood and is selflimiting in the majority of cases.