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1/25. Henoch-Schonlein syndrome associated with human parvovirus B19 primary infection.

    We report a case of Henoch-Schonlein syndrome associated with human parvovirus B19 primary infection. The patient, a 27-year-old Filipino woman, presented with an erythemato-papular-purpuric eruption localized to the lower limbs. General symptoms and signs included fever, hypotension, nausea, vomiting, abdominal pain, inguinal lymphadenopathy and polyarthralgia. Laboratory examinations showed leukocytosis, increase in total serum IgA, proteinuria and haematuria. Circulating IgA immune complexes were also present. The ELISA test for anti-human parvovirus B19 IgM was positive. Histopathological examination revealed a leukocytoclastic vasculitis. This case confirms that also in adult patients, Henoch-Schonlein syndrome may be associated with human parvovirus B19 infection.
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2/25. Acute rheumatic fever associated with Henoch-Schonlein purpura: report of three cases and review of the literature.

    AIM: To describe a possible relationship between Henoch-Schonlein purpura and rheumatic fever. methods: patients with features of both diseases were identified by reviewing the hospital records. medline and reference lists from published articles were used to search for previous reports of the two conditions occuring simultaneously. RESULTS: Three newly described cases, and three previous reports of Henoch-Schonlein purpura associated with rheumatic carditis or chorea were identified. CONCLUSIONS: The coexistence of these two disorders in some patients supports the view that Group A streptococcus may have a pathogenic role in Henoch-Schonlein purpura.
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3/25. Acute infectious purpura fulminans: a 15-year retrospective review of 28 consecutive cases.

    Acute infectious purpura fulminans (AIPF) is a rare syndrome of hemorrhagic infarction of the skin, extremity loss, and intravascular thrombosis. It progresses rapidly and is accompanied by disseminated intravascular coagulation and vascular collapse. The victims often succumb to the disease. Our objective was to investigate the clinical manifestations, outline the clinical course, and delineate factors related to mortality among the patients with AIPF. patients diagnosed with AIPF over a 15-year period were reviewed retrospectively for patient history, comorbid conditions, progression of clinical course, and medical and surgical management. The vast majority of the patients were under the age of 7; however, the disease process can be seen in adults. The overall mortality rate was 43 per cent. The major predisposing factors were history of recent upper respiratory infection, recent surgery or childbirth, young age, and absence of a spleen. The most common clinical manifestations were skin discoloration, disseminated intravascular coagulation, fever, and septic shock. The most common bacteria cultures were neisseria meningitidis, Hemophilus influenzae, and streptococcus pneumoniae. There appears to be a higher mortality in patients who did not undergo a surgical intervention. Compartment syndrome needs to be evaluated early on in the presentation. Rapid diagnosis, intensive care unit management, and prompt surgical consultation and debridement may decrease the mortality.
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4/25. Variable expression of vasculitis in siblings with familial mediterranean fever.

    familial mediterranean fever (FMF) is an autosomal recessive disorder characterized by recurrent and self-limited attacks of serosal inflammation with abdominal pain, chest pain, and arthritis usually accompanied by fever. Different vasculitides such as polyarteritis nodosa (PAN) and Henoch-Schonlein syndrome (HSS) may be associated with FMF. We report two sisters of a Turkish family with FMF who developed distinct vasculitides. The younger sister developed severe PAN with perirenal hematoma at the age of 13 years, the older sister presented with severe HSS and acute renal failure at the age of 19 years. Neither sister developed amyloidosis until the age of 30 years. This observation suggests that early events in the pathogenesis of PAN and HSS are generally quite similar.
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5/25. An idiopathic skin eruption resembling a butterfly rash in a septic patient with disseminated intravascular coagulation following bone marrow transplantation.

    A 31-year-old man who underwent chemotherapy and bone marrow transplantation to treat acute myeloblastic leukemia was admitted to our department complaining of high fever and hypotension. His physical examination revealed warm shock state, eruptions resembling that seen in systemic lupus erythematosus on his face and cyanosis in his fingers. We diagnosed septic shock and idiopathic skin eruption on his face. Following treatment with blood transfusion, anticoagulant, antibiotics, respirator and continuous arteriovenous hemofiltration and dialysis, the patient's condition gradually improved. The eruptions on his face first observed at admission progressed with a worsening of his disseminated intravascular coagulation (DIC), and subsided with an improvement in his DIC. A biopsy of the eruption was taken and pathological findings of the eruption revealed multiple micro-fibrin depositions of the dermis. The skin necrosis in purpura fulminans often begins in the distal extremities. But our patient developed this uncommon skin eruption on his face. patients with an idiopathic skin eruption resembling a butterfly rash in a septic patient should be considered to complicate DIC as in the present case.
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6/25. Henoch schonlein purpura with rheumatic carditis.

    Henoch Schonlein Purpura with acute rheumatic carditis is a rare entity and only few cases have been reported. An 8 year-old-girl presented with abdominal pain, arthralgia and rashes and was diagnosed as a case of Henoch Schonlein Purpura. She was managed conservatively and discharged. She was readmitted after 1 week with abdominal pain, fever and cough. She developed tachycardia with gallop rhythm on the third day of admission and pansystolic murmur of mitral regurgitation. echocardiography showed features of myopericarditis, mild pericardial effusion and mitral regurgitation. She was diagnosed and managed as a case of acute rheumatic carditis.
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7/25. Infantile Henoch-Schonlein purpura.

    We present an infant with generalized palpable purpura, arthritis and fever, whose findings were atypical for classic childhood Henoch-Schonlein purpura (HSP). By describing the clinical symptoms and prognostic differences seen in infants versus school age children, we encourage physicians to be aware of infantile HSP in their differential diagnosis when they encounter a non-toxic infant with generalized purpura.
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8/25. gallbladder involvement of Henoch-Schonlein purpura mimicking acute acalculous cholecystitis.

    BACKGROUND/AIMS: We report the case of a 53-year-old man with fever and severe abdominal pain following an upper respiratory tract infection. methods/RESULTS: The clinical signs, the laboratory markers, and the ultrasound findings were consistent with acute acalculous cholecystitis and he underwent cholecystectomy. Histologically the gallbladder showed vasculitis and the patient developed postoperatively a purpuric rash of the legs with transient ankle arthritis. gastroscopy revealed prepyloric ulcers consistent with vasculitis. The patient was diagnosed as having Henoch-Schonlein purpura. Gastric ulcers as well as arthritis disappeared upon treatment with corticosteroids and proton pump inhibitors. CONCLUSION: Taken together, Henoch-Schonlein purpura can mimic acute cholecystitis and should be considered as a rare differential diagnosis of acute cholecystitis.
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9/25. Henoch-Schonlein purpura associated with pulmonary mycobacterium avium-intracellulare complex.

    A 57-year-old woman presented with bloody sputum and high grade fever. She had been treated for mycobacterium avium-intracellulare complex (MAC). High grade fever slightly decreased and bloody sputum disappeared after two weeks, but low grade fever persisted. After 3 days of recurrence of bloody sputum, she suddenly complained of palpable pururitic lesions on the bilateral lower extremities with bilateral gonalgia. Although there are some reports of direct skin lesions due to MAC, there are no reports of hypersensitivity vasculitis, Henoch-Schonlein purpura, in MAC. It is necessary to consider MAC infection as a potential cause of Henoch-Schonlein purpura.
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10/25. Middle colic artery rupture in a patient with ANCA associated vasculitis: a case report.

    Antineutrophil cytoplasmic antibody (ANCA) positive vasculitides are a heterogeneous group of disorders. They can be disabling multi-organ system conditions. Involvement of small and occasionally medium-sized blood vessels is generally the rule. Recent classification changes and advances in laboratory testing have made it easier to work up a patient suspected of having an ANCA-associated vasculitis. Syndromes can at times overlap, leaving a class of patient that does not fit into one specific group. patients frequently present with a myriad of symptoms ranging from sore throat, fever, athralgias and myalgias, to overt hemorrhage and/or renal failure. The standard treatment remains immunosuppression with steroids, as well as the use of cyclophosphamide, azathioprine, or methotrexate. plasmapheresis and intravenous immunoglobulin (IVIG) are also used. Gastrointestinal complications are a rare manifestation, first described in polyarteritis nodosa and more recently noted in Wegener's granulomatosis. This paper describes a patient with ANCA positive vasculitis who had a spontaneous rupture of the middle colic artery and multiple superior mesenteric aneurisms. It reviews current literature on the ANCA positive vasculitides, including current classification and treatment modalities.
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