Gangrene of the auricle as the fi rst sign of antiphospholipid antibody syndrome (original) (raw)
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Journal of Dhaka Medical College, 2016
Anti-phospholipid antibody syndrome can be defined as the occurrence of venous and arterial thrombosis with or without recurrent miscarriage in association with laboratory evidence of persistent Antiphospholipid antibody / antibody to beta-2-Glycoprotein-1 / anti cardiolipin antibody /lupus anticoagulant (usually associated with SLE). It occurs usually in female who can present with recurrent miscarriage and fetal loss. Anti-phospholipid Antibody can be also found in some autoimmune diseases and post viral infections. Even certain drugs; e.g. phenothiazine, can cause it. Arterial thrombosis may lead to peripheral limb ischemia, stroke, and myocardial infarct. And venous thrombosis may be found in the form of DVT, pulmonary embolism & thrombosis in vessels supplying the abdominal organ.J Dhaka Medical College, Vol. 24, No.2, October, 2015, Page 152-155
Journal of the American Academy of …, 2007
The antiphospholipid syndrome (APS) is an autoimmune disorder characterized by the occurrence of arterial and/or venous thromboembolic events and obstetric complications in the setting of circulating antiphospholipid antibodies. Dermatologic manifestations are commonly seen in APS with almost half of the patients exhibiting varied conditions such as ulceration, splinter hemorrhages, and livedo reticularis. In this paper, we report the case of a 12-year-old boy who was diagnosed with APS after presenting with livedo reticularis and positive antiphospholipid antibodies. We discuss the difficulty of diagnosing APS in patients presenting solely with dermatologic complaints, as these skin manifestations are not specific enough for APS to be included in the Sapporo diagnostic criteria. Proposed revisions to the Sapporo criteria to increase its specificity and sensitivity are also addressed.
The antiphospholipid antibody syndrome: diagnosis, skin manifestations and current therapy
Clinical and experimental rheumatology
Antiphospholipid antibody syndrome is characterized by venous and/or arterial thrombosis and/or pregnancy morbidity associated with antiphospholipid antibodies (aPL), such as anticardiolipin antibodies, anti beta 2 glycoprotein I antibodies and positive lupus anticoagulant test. This syndrome may potentially affects any organ system including the skin. Livedo reticularis is the most frequently observed cutaneous lesion; other lesions, by order of frequency, are ulcerations, digital gangrene, subungueal splinter hemorrhages, superficial venous thrombosis, thrombocytopenic purpura, pseudovasculitic manifestations, extensive cutaneous necrosis and primary anetoderma. Skin lesions are more frequently observed in the catastrophic antiphospholipid syndrome, characterized by widespread microvascular occlusions involving multiple organs simultaneously. Patients with antiphospholipid associated thrombosis should receive long-term oral anticoagulants. The intensity of anticoagulation should b...
The antiphospholipid antibody syndrome: a case report
International Medical Case Reports Journal, 2012
Antiphospholipid antibody syndrome is defined by the presence of thromboembolic complications and/or pregnancy morbidity in the presence of persistently increased titers of antiphospholipid antibodies. Its clinical presentation can be diverse and any organ can be involved, with a current impact in most surgical and medical specialties. The authors present the case of a 43-year-old man who, over a 13-year period of follow-up, presented with thrombosis of the mesenteric vein, inferior vena cava, and axillary and subclavian veins in a setting where diagnostic and therapeutic options are limited and costly. Through this case report, the authors aim to describe the evolution of this complex pathology, which to date has not been described in the authors' milieu -probably because of its challenging diagnosis and the limited treatment options available. The authors conclude that clinicians need to have a high index of suspicion of APS in patients who present with a thrombotic episode -clinicians should investigate for the presence of antiphospholipid antibodies, as early diagnosis may influence the course of the disease. Furthermore, resources for the detection of antiphospholipid antibodies should be made readily available in resource-limited settings. Finally, patient education on the importance of drug compliance, periodic monitoring, and prevention of thrombosis is indispensable, especially as mortality could be associated with the effects of vascular thrombosis and/or the effects of bleeding due to anticoagulants.
The Hematologic Manifestations of the Antiphospholipid Syndrome
Blood Reviews, 2008
Various hematological pathologies have been described in association with antiphospholipid syndrome (APS). Thrombocytopenia is frequently found in APS patients, its incidence has ranged from 22-42% in different series, it is usually moderate (>50 · 10 9 /L) without clinical manifestation and requires no intervention. A high percentage of patients with isolated idiopathic thrombocytopenic purpura have antiphospholipid antibodies, however the pathogenetic role and the clinical importance of these antibodies in this condition is still not clear. Other hematological manifestations reported in association with APS include: bone marrow necrosis, and various thrombotic microangiopathic syndromes such as: thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome, HELLP (hemolysis, elevated liver enzymes, low platelet) syndrome, and catastrophic APS. A high index of suspicion is needed for the early recognition and treatment of these conditions.