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1/13. Exacerbation of antiphospholipid antibody syndrome after treatment of localized cancer: a report of two cases.

    patients with malignancy often present with a variety of coagulation abnormalities which may ultimately lead to recurrent arterial and venous thromboses. Recently the presence of antiphospholipid antibodies in cancer patients has been proposed as one of the potential mechanisms promoting hypercoagulability. Here we report two consecutive patients with localized tumors, one suffering from breast cancer and another presenting with colorectal cancer, who experienced dramatic exacerbation of the antiphospholipid antibody syndrome (APAS) within 4 weeks after surgery. In the first patient who had also received one course of adjuvant chemotherapy, major ischemic stroke and recurrent venous thromboembolism were paralleled by the development of ulcerative livedoid vasculitis and pancytopenia, constituting the diagnosis of systemic lupus erythematosus with secondary APAS. In the second patient, progressive thrombotic occlusion of the superior and inferior vena cava was associated with bilateral pulmonary embolism, acute renal failure, and disabling soft tissue edema. Although not fulfilling the classic criteria of "catastrophic" APAS, the clinical features were life threatening and appeared to be refractory to oral anticoagulation with phenprocoumon. In addition, a diagnosis of Trousseau's syndrome was unlikely due to missing evidence of gross metastatic disease. Besides a suggested treatment strategy comprising high doses of low-molecular-weight heparin, potential pathogenic mechanisms are discussed in consideration of a recently proposed "thrombotic storm," which may cause multiple thromboses after an initial provocation in patients with known hypercoagulability.
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ranking = 1
keywords = livedo
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2/13. Reticulate and stellate acral pigmentation associated with systemic lupus erythematosus and high titers of circulating anticardiolipin antibodies: a possible association with acral microlivedo.

    A Hispanic man with a twenty-eight year history of systemic lupus erythematosus (SLE) and a high titer of anticardiolipin IgG antibodies was noted to have reticulate and stellate acral pigmentation. The patient reported that hand swelling and erythema developed soon after the diagnosis of SLE was established. This episode resolved quickly without recurrence or immediate sequelae. We postulate that this eruption was related to SLE and anticardiolipin antibodies. Reticulate and stellate acral pigmentation should be considered a possible manifestation of SLE and high titers of anticardiolipin antibodies, or a consequence of therapy.
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ranking = 4
keywords = livedo
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3/13. Systemic lupus erythematosus with unusual maculopapular and erosive cutaneous lesions.

    In 2002, a 38-year-old woman was referred to the department of dermatological sciences for the evaluation of erythematous-infiltrated lesions with prominent purpuric and erosive components distributed on her face, upper chest, and extensor surfaces of the limbs, which developed over a 3-month period. In 1997 the patient suffered from polyarthralgia (proximal interphalangeal,knee, wrist, and metacarpophalangeal joints) associated with morning stiffness. In 1999 she was admitted to a psychiatric unit for depression with episodes of lipothymia. In the same year, since she developed diffuse and persistent urticarial manifestations with angioedema and livedo reticularis of the limbs in association with anticardiolipin antibodies, vasculitis was proposed. Upon examination, erythematous-infiltrated and erosive lesions in association with serohemorrhagic crusts were present on the face,neck, chest, upper trunk, and extensor surfaces of the upper limbs and thighs (Figures 1-3). Telangiectasias were especially evident on the cheeks, where prominent edema conferred her face a moon-like appearance (Figure 1). Laboratory investigations disclosed microcytic anemia, elevated erythrocyte sedimentation rate, proteinuria (30 mg/dL), positive antinuclear antibodies (1:80) with diffuse fluorescence pattern, lowered complement levels (C3: 31 mg/dL, C4: 3 mg/dL), circulating immunocomplexes binding Clq, and high titers of anti-TSH receptor antibodies. Indirect immunofluorescence was negative for the detection of anti-basement membrane zone antibodies. Histological examination of the lesional skin of the shoulder documented epidermal atrophy, marked vacuolar degeneration of the basal cell layer, colloid bodies, and a perivascular lymphohistiocytic infiltration of the upper and deep dermis (Figure 4). Direct immunofluorescence performed on affected skin showed junctional granular deposition of IgG, C3,and Clq in association with perivascular C3 and Clq deposits in the upper dermis; direct immunofluorescence on sun-protected,non-lesional skin showed similar immunological deposition both at the basement membrane zone and in the perivascular dermis.Based upon the clinical, histologic, and immunopathologic findings, we proposed the diagnosis of systemic lupus erythematosus with maculopapular and erosive skin lesions. The patient was treated with methylprednisolone IV (pulse therapy, 250 mg) tapered over 10 days and later continued p.o. After a few weeks, significant improvement of cutaneous manifestations was noted.
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ranking = 8.8556540756648
keywords = livedo reticularis, reticularis, livedo
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4/13. Systemic lupus erythematosus and lymphoma: association with antiphospholipid syndrome.

    A woman with a history of polyarthralgias appeared to develop systemic lupus erythematosus and lymphoma simultaneously. The diagnosis of the concurrent lymphoma was made on biopsy of a left axillary lymph node. Generalized lymphadenopathy, splenomegaly and pruritus had given rise to suspicion of an underlying lymphoma. The lymphoma responded well to chemotherapy. Her condition was further complicated by an arterial occlusion involving the right 2nd toe which was eventually amputated, transient ischemic attacks (amaurosis fugax), livedo reticularis and thrombocytopenia which were accompanied by elevations of IgM anticardiolipin antibodies and a biological false test for syphilis. The lupus anticoagulant test was not performed as she was given anticoagulation therapy.
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ranking = 8.8556540756648
keywords = livedo reticularis, reticularis, livedo
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5/13. anterior spinal artery syndrome in systemic lupus erythematosus.

    We present a patient with systemic lupus erythematosus who developed an anterior spinal artery syndrome (ASAS) in association with livedo reticularis, leg ulcerations and thrombocytopenia. Low serum titres of anticardiolipin antibodies were detectable throughout the course of this disease. The patient recovered from the first episode of ASAS under corticosteroid treatment but remained paralytic after a second episode. Repeated magnetic resonance imaging of the spinal cord failed to show altered signal intensity.
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ranking = 8.8556540756648
keywords = livedo reticularis, reticularis, livedo
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6/13. Cutaneous manifestations associated with the presence of the lupus anticoagulant. A report of two cases and a review of the literature.

    Two patients with the lupus anticoagulant exhibited unusual cutaneous manifestations. They both fulfilled four criteria for systemic lupus erythematosus and had experienced deep venous thrombosis. The first patient suffered from a leg ulcer that resembled a pyoderma gangrenosum. The second patient presented erythematous and purplish macules on the fingertips. The histologic studies showed only microthrombosis in the dermal vessels without vasculitis, although such lesions in systemic lupus erythematosus are usually attributed to vasculitis. The association of these cutaneous lesions with lupus anticoagulant has never been reported. It is likely that this association is not fortuitous. After a review of the literature, it seems possible to individualize a new syndrome characterized by the presence of a subgroup of antiphospholipid antibodies. Thrombosis, spontaneous abortions, neurologic manifestations, pulmonary hypertension, positive results of a Coombs' test, and thrombocytopenia can be included in this syndrome, which overlaps with systemic lupus erythematosus. Certain cutaneous symptoms are associated with the presence of lupus anticoagulant or other antiphospholipid antibodies: leg ulcers, distal cutaneous ischemia, widespread cutaneous necrosis, and livedo. They can be considered as the dermatologic manifestations of this syndrome.
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ranking = 1
keywords = livedo
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7/13. Recurrent stroke and multi-infarct dementia in systemic lupus erythematosus: association with antiphospholipid antibodies.

    Four patients with recurrent stroke and multi-infarct dementia are presented in whom the dementia was progressive and severe. Three of the patients developed the dementia during the course of an illness which was punctuated by repeated episodes of cerebral infarction demonstrated by computed tomographic (CT) scans. The fourth patient presented with an illness dominated by progressive and deteriorating higher mental functions, which culminated in a major stroke 18 months later. Three patients fulfilled the American Rheumatism association (ARA) criteria for the classification of systemic lupus erythematosus, the fourth had a 'lupus-like' disease. All had livedo reticularis, severe migraines, and also demonstrated antibodies to phospholipids. All four patients suffered deep vein thromboses.
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ranking = 8.8556540756648
keywords = livedo reticularis, reticularis, livedo
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8/13. Diagnostic and therapeutic problems in two patients with antiphospholipid antibodies, heart valve lesions, and transient ischaemic attacks.

    Two young women (aged 32 and 25 years) with systemic lupus erythematosus and heart valve lesions in association with antiphospholipid antibodies are presented. In addition to the presence of the 'lupus anticoagulant' and false positive Venereal disease research Laboratory (VDRL) tests, both patients had high levels of IgG anticardiolipin antibodies. The first patient additionally had contraceptive induced chorea, chorea gravidarum, seven miscarriages, livedo reticularis, pulmonary embolism, and thrombocytopenia and developed culture negative endocarditis as well as hypertension. The second patient, who had presented with hypertension, developed aortic and mitral regurgitation, suspected myocarditis, manifested transient ischaemic attacks, and responded well to anticoagulation and steroid treatment.
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ranking = 8.8556540756648
keywords = livedo reticularis, reticularis, livedo
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9/13. Anticardiolipin antibodies, livedo reticularis, and major cerebrovascular and renal disease in systemic lupus erythematosus.

    Increased levels of IgG anticardiolipin antibodies (ACA) were found in 23 of 98 patients (23%) with systemic lupus erythematosus. Increased levels of IgM ACA were found less frequently (5%). All four patients with major cerebrovascular events had increased IgG ACA, including the two with highest levels, both of whom were men. Six of nine patients with livedo reticularis had increased IgG ACA. In turn, this clinical feature was associated with cerebrovascular disease in two and renal disease in another six. IgG ACA were not associated with other thromboembolic events, thrombocytopenia, serum IgG, or autoantibodies to Ro(SSA), La(SSB), U1RNP, Sm, or double or single stranded dna.
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ranking = 44.278270378324
keywords = livedo reticularis, reticularis, livedo
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10/13. livedo reticularis and cerebrovascular accidents.

    livedo reticularis is a clinical pattern of cutaneous vascular involvement that can be seen in association with many diseases. The association of livedo reticularis and cerebrovascular accidents has been reported rarely in the past. We report on a 52-year-old woman with marked generalized livedo reticularis and cerebrovascular accidents who exhibited endarteritis obliterans without other pathologic changes on histologic examination of the skin. This constellation of findings suggest a primary vasculopathy with rather extensive involvement which merits our attention and further study.
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ranking = 26.492017129272
keywords = livedo reticularis, reticularis, livedo
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