Livedo Reticularis

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In recent years, it has been recognized that livedo reticularis is the manifestation of the presence of antiphospholipid antibodies in patients with SLE [(secondary antiphospholipid syndrome (APS)]. One study indicates that 17% of 66 patients with SLE demonstrated the presence of livedo reticularis and that 81% of these patients demonstrated the presence of antiphospholipid antibodies (Yasue 1986). The APS can also occur in the absence of evidence of SLE and is termed "primary APS."

Patients with SLE possessing antiphospholipid antibodies have a disease process that is characterized by recurrent arterial and venous thrombosis, thrombocytope-nia, increased fetal wastage late in the first trimester, valvular heart disease, Libman-Sachs endocarditis, and pulmonary hypertension. In addition to the livedo pattern on the skin, these patients may demonstrate acrocyanosis, small vessel infarction predominantly on the lower extremities producing atrophie blanche-like lesions. On unusual occasions, cutaneous infarction may occur, simulating Degos' disease.

The most common serious systemic sequelea associated with livedo reticularis is repeated central nervous system infarction producing neurologic defects and frequently resulting in postinfarction dementia (Sneddon's syndrome) (Levine et al. 1988, Sneddon 1965).

The APS is serologically characterized by a heterogeneous group of autoanti-bodies, which interfere with intrinsic anticoagulant macromolecules located on blood vessel endothelial cell surfaces (Levin 2002, Roubey 1996). These antibodies are most commonly directed at negatively charged phospholipids complexed with P2 glycoprotein I (apolipoprotein H). Epitopes formed by this complex of negatively charged phospholipid^ glycoprotein I or epitopes localized only to P2 glycoprotein I are the pathologic antigens. Other possible pathophysiologic mechanisms occurring in the APS are listed in Table 7.2.

Table 7.2. Procoagulant effects of antiphospholipid antibodies on coagulation (see Levin 2002)

Inhibition of the activated protein C pathway Up-regulation of the tissue factor pathway Inhibition of antithrombin III activity Disruption of annexin V shield on membranes Inhibition of anticoagulant activity of |32 glycoprotein I Inhibition of fibrinolysis

Enhanced expression of adhesion molecules by endothelial cells and adherence of neutrophils and leukocytes to endothelial cells Activation and degranulation of neutrophils Potentiation of platelet activation Enhanced platelet aggregation Enhanced binding of |32 glycoprotein I to membranes Enhanced binding of prothrombin to membranes

It should be recognized that antiphospholipid antibodies occur in chronic infections such as syphilis and leprosy and with drugs such as chlorpromazine. These autoantibodies, however, are directed against the phospholipid protein alone and not P2 glycoprotein I and are unassociated with a hypercoagulable state.

The antiphospholipid antibodies are detected by two techniques: lupus anticoagulant and anticardiolipin assays. Lupus anticoagulant activity is detected by the use of one of a variety of phospholipids-dependent in vitro coagulation assays. These assays include activated partial thromboplastin time, dilute activated thromboplastin time, colloidal silica clotting time, kaolin clotting time, and the Russel viper venom test. The anticoagulant activity detected in patients with the anti-APS is not corrected by mixing the patient's plasma with fresh normal plasma. However, the prolonged coagulation time is corrected by excess phospholipid or platelets that have been frozen and then thawed.

The second test commonly used to detect the APS measures anticardiolipin antibodies. Anticardiolipin antibodies are generally detected by a solid-phase assay in which cardiolipin-coated plates in the presence of bovine serum P2 glycoprotein I are reacted with the patient's serum. In addition, recent assays to detect anti-P2 glycopro-tein I antibodies have been developed.

These two assays (lupus anticoagulant and anticardiolipin) are concordant in most cases. However, it has been recognized that the APS can occur in patients possessing antibodies directed against activated protein C, protein S, thrombin III, and annexin V (for a review, see Levine 2002). All these antibodies are capable of inducing a hypercoagulable state in patients with SLE. Each of these antibody systems produces a positive lupus anticoagulant assay, but each fails to demonstrate anticardiolipin or anti-P2 glycoprotein I activity (anticardiolipin negative).

Treatment of APS has been successfully accomplished using Warfarin, maintaining an anticoagulant activity equal to or greater than an international normalization ratio of 3 (Khamashta et al. 1995).

Table 7.3. Provocative phototesting in lupus erythematosus (see Kuhn et al. 2001)

Table 7.3. Provocative phototesting in lupus erythematosus (see Kuhn et al. 2001)


74 62 63 104 20


74 62 63 104 20

CLE, chronic lupus erythematosus (hypertrophic lupus erythematosus, lupus erythematosus profundus, chilblain lupus erythematosus); DLE, discoid lupus erythematosus; LET, lupus erythematosus tumidus; SCLE, subacute cutaneous lupus erythematosus; SLE, systemic lupus ery-thematosus.

a Cumulative positive results from ultraviolet (UV)A-positive, UVB-positive, and UVA- + UVB-positive patients.

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