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1/119. Cutaneous necrosis as a terminal paraneoplastic thromboembolic event in a patient with non-Hodgkin's lymphoma.

    Thrombotic complications in non-Hodgkin's lymphoma often originate in the large veins. We describe a patient with refractory advanced high-grade lymphoma who presented with the rare complication of extensive cutaneous necrosis due to thrombosis of dermal vessels; there was also a recent new peak of monoclonal IgM-kappa protein. Direct immunofluorescence demonstrated immune deposits with complement in the dermal vessel wall. Based on these observations and on published data, we suggest that these complexes were the trigger for the thrombotic events and that the monoclonal IgM acted as xenoreactive antibodies, initiating a cascade of events. The first step of this cascade was activation of the complement and the membrane attack complex, which caused secretion of IL-1 alpha by endothelial cells, followed by overexpression of tissue factor on the surface of the dermal vessel wall endothelium. Dermal vessel thrombosis was the final event in this cascade.
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2/119. Numerous and large Bier's spot associated with pregnancy.

    We report a patient with anemic macules over cyanotic skin which started in the 8th month of a normal pregnancy. We thought that these lesions were close to Bier's spot description although it has never been described during pregnancy. The relationship with pregnancy is likely because skin lesions regress in post-partum. It probably represents an exaggerated physiological response of small vessels to venous hypertension observed during pregnancy in predisposed women.
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3/119. Annular vasculitis in association with sarcoidosis.

    We report a case of cutaneous vasculitis with annular features in association with sarcoidosis. A 36-year-old woman presented with fever, polyarthralgias, erythema nodosum, bilateral hilar lymphadenopathy, and induration of a long-standing scar on the face. In addition, she developed annular, erythematous, and purpuric patches over her thighs and buttocks that were histologically characterized by a small vessel leukocytoclastic vasculitis. The presence of circulating immune complexes in the early stages of sarcoidosis might be related to the occurrence of the vascular damage.
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keywords = vessel
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4/119. Excess tissue friability during CO2 laser vaporization of nodular amyloidosis.

    BACKGROUND: Nodular amyloidosis is a rare cutaneous disorder for which many treatments have been described, including carbon dioxide (CO2) laser vaporization. OBJECTIVE: We describe the intraoperative tissue effects encountered during successful treatment of nodular amyloidosis with CO2 laser surgery. methods: Two nodules of cutaneous amyloid on the nose of a patient were vaporized with a defocused, continuous-wave CO2 laser. RESULTS: The tissue was found to be highly friable and hemostasis was difficult to achieve during the surgery. These clinical observations were correlated with amyloid infiltration of the dermis and blood vessels on preoperative histologic examination. CONCLUSION: CO2 laser vaporization of nodular amyloidosis may be associated with prominent tissue friability and poor hemostasis.
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ranking = 3.2804526196737
keywords = blood vessel, vessel
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5/119. Acquired cutaneous lymphangiectasia in a patient with cirrhotic ascites.

    BACKGROUND: Lymphangiectasia results from acquired dilation of lymphatic vessels. Areas of skin affected by obstruction or destruction of lymphatic drainage are said to be prone to the development of lymphangiectasia. Cirrhosis is a cause of alterations of lymph flow. methods: Case report. RESULTS: We report a case of acquired, late-onset, lymphangiectasia associated with alcoholic hepatic cirrhosis. Lesions were scattered over the right, lower, anterior abdominal wall, a region that is drained by a common group of lymphatic vessels that were probably disordered. CONCLUSION: We think that this is the first reported case of lymphangiectasia associated with altered lymph flow in cirrhosis and ascites.
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keywords = vessel
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6/119. Tumoral calcinosis: a case report with an electron microscopic study.

    A 68-year-old woman developed large subcutaneous masses on her abdomen and thighs after a bruise sustained in a traffic accident. She had severe pain when sitting up straight. Histological examination revealed calcified tissues in the entire dermis of the injured areas. On electron microscopy, crystalline materials were observed in the dermis, which seemed to be formed by the deposition of hydroxyapatite on unusual proteoglycan. In a vessel wall, a thick, layered basement membrane was observed. This suggests that vascular injury and subsequent hypoxia play a role in the process of calcinosis. We performed a partial resection with good results in alleviating the patient's pain.
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7/119. A unique case of a benign disseminated angioproliferation combining features of Kaposi's sarcoma and diffuse dermal angioendotheliomatosis.

    A female patient undergoing chronic hemodialysis had disseminated, violaceous, and partly ulcerated plaques develop on the trunk. Lesions had erupted simultaneously over a period of 4 weeks and resolved within 5 months after steroid treatment. By histopathology, the papillary dermis was densely filled with blood vessels lined by a single layer of differentiated endothelial cells, a growth pattern resembling diffuse dermal angioendotheliomatosis. In some areas, endothelial cells were spindle shaped and formed discontinuous lumina. Red blood cells were interspersed within these slits, giving the lesions a kaposiform appearance. By immunohistochemistry, endothelial cells reacted with the antibodies anti-von willebrand factor, anti-CD31, and anti-CD34 and with the lectin ulex europaeus-1. The course of the disease combined with the unusual histopathology makes this case a unique form of a benign disseminated kaposiform angioproliferation.
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ranking = 3.2804526196737
keywords = blood vessel, vessel
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8/119. Waldenstrom's macroglobulinaemia presenting as reticulate purpura and bullae in a patient with hepatitis b virus infection.

    Cutaneous manifestations of Waldenstrom's macroglobulinaemia (WM) include purpura, ulcers, urticaria, leukocytoclastic vasculitis, and immunobullous dermatoses. No association has been reported previously of WM and hepatitis b virus (HBV) infection. A 40-year-old female HBV carrier was admitted to hospital because of generalized oedema, oliguria, haematuria, hypertension, fever and blood-tinged sputum. Cutaneous manifestations included generalized petechiae, palpable purpura mainly on the legs, multiple necrotic ulcerations and gangrenous changes on the toes, and necrotic, giant confluent reticulate purpura on the trunk surmounted by several tense bullae. Laboratory investigations revealed monoclonal gammopathy of IgM kappa type (6.7 g/L), membranoproliferative glomerulonephritis associated with HBV infection, Bence Jones proteinuria, and an increased number of abnormal plasmacytoid cells in the bone marrow. Pathologic examination demonstrated immune complex-mediated vasculitis with deposits of IgM in the walls of dermal vessels and secondary subepidermal bulla formation. HBV infection may have caused WM or modified the clinical course in this fatal case.
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ranking = 0.25
keywords = vessel
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9/119. Subcutaneous angiolymphoid hyperplasia (Kimura disease). Report of a case.

    A subcutaneous mass removed from the cheek showed histologic features of subcutaneous angiolymphoid hyperplasia (Kimura disease) at its early stage. The condition shows a wide spectrum of pathologic changes. At its early stage, the main findings consist of active vascular proliferation with plump endothelial cells and varying degrees of lymphocytic, histiocytic, and eosinophilic infiltration. The lesion at its later stage features hyperplastic blood vessels with inconspicuous endothelial cells, well-formed lymphoid follicles, and varying degrees of lymphocytic and eosinophilic infilitration. Blood eosinophilia is frequently seen. review of the literature and study of our own case strongly suggest that this disease is a distinct clinical and pathologic entity.
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ranking = 3.2804526196737
keywords = blood vessel, vessel
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10/119. Cutaneous lymphatic amyloid deposits in "Hungarian-type" familial transthyretin amyloidosis: a case report.

    Multiple transthyretin (TTR) mutations have recently been identified and implicated in the development of familial systemic amyloidoses, but early diagnosis of these disorders is still largely unresolved. We investigated the presence and tissue distribution of TTR-derived amyloid in skin biopsies of a 59-year-old woman carrying the "Hungarian-type" mutation of TTR (Asp18Gly). Clinical symptoms involved severe central nervous system dysfunction without signs of polyneuropathy, also referred to as the "central form" of TTR-related systemic amyloidosis. skin biopsy was also evaluated as a tool in order to diagnose this type of TTR amyloidosis. biopsy samples were collected from the infra-axillary region. light microscopy using congo red and polarized light was used to diagnose amyloid deposits. Subsequently, electron microscopic analysis was performed to correlate the amyloid deposits with vicinal dermal structures. The amyloid class was determined by means of immunocytochemistry. TTR amyloid was primarily localized to lymphatic microvessels in the present case, whereas arterioles were devoid of TTR amyloid deposits. In addition, the well-known association of TTR amyloid with neural structures along the erector pilorum and around the sebaceous and serosal (sweat) glands was also evident. Electron microscopic analysis of amyloid deposits revealed characteristic amyloid fibrils that were irregular in shape, and exhibited a heterogeneous density and a random deposition pattern. Immunocytochemistry confirmed the cutaneous accumulation of TTR amyloid. In conclusion, amyloid deposits were abundantly present in the skin of a patient with "Hungarian-type" TTR amyloidosis; skin biopsy seems to be appropriate for the diagnosis of this disorder. We showed that besides the erector pilorum, sweat glands and nerve terminals, lymphatic microvessels are also severely infiltrated by TTR amyloid. Whether these pathological alterations can exclusively be found in "Hungarian-type" TTR amyloidosis should still be investigated. If such changes are not specific for the Asp18Gly mutation, they may be considered as diagnostic markers for "central" TTR amyloid disorders.
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