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1/46. Ischemic necrosis of bile ducts complicating Schonlein-Henoch purpura.

    Gastrointestinal complications of Schonlein-Henoch purpura are frequent and sometimes severe. However, there seem to be no reports of liver involvement. A child is described in whom Schonlein-Henoch purpura was complicated by bile duct lesions, resulting in biliary cirrhosis and requiring liver transplantation. At surgical removal, the liver had lesions of bile ducts and of adjacent small blood vessels in the hilum, very similar to those complicating hepatic artery thrombosis after liver transplantation. These findings suggest that Schonlein-Henoch purpura can be complicated by vasculitis of the peribiliary vessels resulting in ischemic necrosis of the bile ducts. Schonlein-Henoch purpura can be added to the list of causes of ischemic cholangiopathies.
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2/46. Acute infantile hemorrhagic oedema.

    Acute infantile hemorrhagic oedema (AIHO) was first described in 1913 but, despite frequent reports in the European literature, it is not well recognized in the English language literature. It is considered by many to be a variant of Henoch Schonlein Purpura (HSP) because of similarities in cause and histopathology. However, because of the benign nature of this condition and frequent absence of IgA associated with HSP, it may be sensible to consider this as a distinct variety of cutaneous small vessel vasculitis (CSVV). We report this case to highlight the condition and emphasize its benign nature.
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3/46. Duodenojejunitis: is it idiopathic or is it Henoch-Schonlein purpura without the purpura?

    BACKGROUND: Henoch-Schonlein purpura is a small-vessel vasculitic disease that most often affects the skin. Gastrointestinal manifestations have been well described, including duodenojejunal inflammation (DJI). methods: Four children with DJI and clinical features of HSP are described, in whom the rash was either not present or appeared atypically late in the illness. RESULTS: The characteristic rash did not develop in three children, and it developed much later in one. The patients (three boys and one girl) were aged between 7 and 9 years (mean, 7.5 years). growth characteristics were normal. In all patients, pain occurred acutely with colicky abdominal pain in the spring or fall of the year, and all stools were positive for occult blood. No infectious cause was identified. Upper gastrointestinal endoscopic examinations demonstrated significant visual and histologic duodenitis in a pattern consistent with previous reports in children with known HSP. factor xiii activity was absent. immunoglobulin a levels were increased in three of four children. All children made a prompt recovery with the administration of intravenous glucocorticoids. In one child, the characteristic rash of HSP developed 18 weeks after the initial examination. CONCLUSION: Duodenojejunal inflammation may be the primary manifestation of HSP, even in the absence of the characteristic rash.
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4/46. propylthiouracil-induced antineutrophil cytoplasm antibody-positive anaphylactoid purpura-like vasculitis--a case report.

    propylthiouracil (PTU), a drug commonly used for treatment of hyperthyroidism, is associated with various rare side effects. Antineutrophil cytoplasm antibody (ANCA)-positive vasculitis is a relatively unusual complication among them. The pathogenesis of ANCA-positive vasculitis during PTU therapy is still obscure. We present the case of a 12-year-old boy who developed ANCA-positive vasculitis during PTU therapy for Graves' disease. His symptoms and signs were indistinguishable from anaphylactoid purpura, a common small-vessel vasculitis in children. The clinical manifestations improved after discontinuation of PTU and immunosuppressant treatment. He remained symptom-free at 11-months follow-up.
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5/46. Free-tissue transfer for limb salvage in purpura fulminans.

    A series of 13 patients is described to demonstrate the experience of the authors with free-tissue transfer for limb salvage in patients with purpura fulminans. A total of seven free-flap procedures were performed, with a loss of flap in one patient. The flaps were used for lower-extremity salvage in six patients and for upper-extremity salvage in one. purpura fulminans is a devastating illness caused by endotoxin-producing bacteria such as meningococcus and pneumococcus. Clotting derangements and systemic vasculitis often lead to widespread tissue necrosis in the extremities. Local tissue is usually not available to cover vital structures in these complex wounds. In these situations, free-tissue transfer is necessary to achieve limb salvage. Microsurgical reconstruction in patients with purpura fulminans is a formidable challenge. Because of high platelet counts and systemic vasculitis, successful microvascular anastomosis is difficult. Abnormally high platelet counts persist well into the subacute and chronic phases of the illness. Pretreatment with antiplatelet agents before microvascular surgery may be beneficial. The systemic nature of the vascular injury does not permit microvascular anastomosis to be performed outside the "zone of injury." Extensive vascular exposure, even at a great distance from the wound, does not reveal a disease-free vessel. The friable intima is difficult to manage with a standard end-to-side anastomosis, but conversion to end-to-end anastomosis may salvage free-tissue transfers in cases in which intimal damage is too severe to sustain a patent anastomosis. patients often have peripheral neuropathies caused by the underlying disease; however, this resolves with time and is not a contraindication to limb salvage.
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6/46. review of management of purpura fulminans and two case reports.

    purpura fulminans (PF) is a haemorrhagic condition usually associated with sepsis or previous infection. Features include tissue necrosis, small vessel thrombosis and disseminated intravascular coagulation. Gram-negative organisms are the commonest cause of the acute infectious type, which is often associated with multi-organ failure. An idiopathic variety, however, is often confined to the skin. The mortality rate has decreased with better treatment of secondary infections, supportive care and new treatments, but it remains a disabling condition often requiring major amputations. We describe two cases and review the various treatments for this condition.
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7/46. Schonlein-Henoch purpura during pregnancy with successful outcome for mother and newborn.

    BACKGROUND: Schonlein-Henoch purpura is a systemic vasculitis that affects vessels of a small caliber and rarely reported in the literature. CASE PRESENTATION: We report on a 35-year-old woman who developed palpable purpura with necrotizing cutaneous lesions on the lower limbs at 27 weeks of gestation. She also complained of epigastric pain and arthralgias. Histologic examination of a skin biopsy showed leukocytoclastic vasculitis with intravascular fibrin thrombi. The direct immunofluorescence analysis evidenced vascular deposits of IgA and C3 in the upper and mid-dermis. These findings were consistent with Schonlein-Henoch purpura. There was no evidence of renal involvement or placental dysfunction. The patient was treated with low-dose oral corticosteroids and a healthy infant was delivered by cesarean section. Examination of the placenta and the navel string disclosed no signs of vasculitis or infarction. CONCLUSION: Schonlein-Henoch purpura is rarely reported in pregnancy. Treatment with orally administered corticosteroids may lead to a beneficial outcome for mother and newborn.
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8/46. Henoch-Schonlein purpura secondary to subacute bacterial endocarditis.

    Henoch-Schonlein purpura (HSP), a systemic, small-vessel vasculitic syndrome, is characterized by a nonthrombocytopenic purpuric rash, arthralgia, abdominal pain, and nephritis. These signs and symptoms may occur in any order, and not all are necessary for the diagnosis. Although most common in 4- to 7-year-olds, HSP is well documented in adults and is often preceded by a history of mucosal-based infections, especially of the upper respiratory tract. We report a case of HSP that occurred coincident with the onset of subacute bacterial endocarditis (SBE) in an otherwise healtny 41-year-old white woman. The patient presented with a purpuric rash and arthralgia and was found to have left-sided streptococcal SBE. She subsequently developed abdominal pain and immune complex glomerulonephritis. The bacterial endocarditis was treated with antibiotics and mitral valve replacement, followed by a spontaneous resolution of the associated signs and symptoms of HSP.
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9/46. Adult Henoch-Schonlein purpura with glomerulonephritis and paroxysmal nocturnal haemoglobinuria: an uncommon association.

    Henoch-Schonlein purpura (HSP) is considered to be a small blood vessel systemic vasculitis. Numerous microorganisms have been implicated in triggering HSP. We describe an unusual case of HSP with glomerulonephritis and renal failure requiring haemodialysis in a young adult man who subsequently developed paroxysmal nocturnal haemoglobinuria (PNH) with several haemolytic episodes. bacterial infections, especially those of the respiratory and urinary tract, might trigger both the diseases.
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keywords = blood vessel, vessel
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10/46. Adult purpura fulminans associated with staphylococcal infection and administration of colony-stimulating factors.

    purpura fulminans (PF) is a rare syndrome of progressive haemorragic necrosis due to disseminated intravascular coagulation (DIC) and dermal vascular thrombosis leading to purpura and tissue necrosis. PF is more often associated with either a benign infection or a severe sepsis. Rarely, it has been related to drug intake. We report the case of a 24-year-old female patient who suffered from staphylococcal sepsis and pancytopenia, for which she was treated with antibiotics, granulocyte-colony stimulating factor (G-CSF) and granulocyte/macrophage CSF (GM-CSF). Two days after the last GM-CSF dose, she developed widespread necrotic plaques with erythematous borders and purpura in the breast, arms and legs. Coagulation tests indicated DIC and a skin biopsy showed fibrin thrombi in the superficial dermal vessels. The patient totally recovered after removal of the necrotic tissues and application of skin autografts. Although staphylococcal infection was most probably involved in the development of PF, a role of CSF cannot be excluded in this case.
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