Protein S deficiency revealed by skin necrosis in a patient with lupus

Lupus ◽  
2019 ◽  
Vol 28 (7) ◽  
pp. 903-905 ◽  
Author(s):  
A Plana-Pla ◽  
I Bielsa Marsol ◽  
C Ferrandiz Foraster

Protein S deficiency is rare in systemic lupus erythematosus (SLE) and is generally associated with the presence of antiphospholipid (APL) antibodies. Lack of protein S can cause skin necrosis, but when it does it is generally in response to warfarin exposure. In this article, we describe the case of a patient who had not received warfarin and without APL antibodies who developed extensive skin necrosis due to protein S deficiency. It is important to investigate protein S deficiency in patients with lupus and extensive skin ulcers as it is a sign of arterial thrombosis and venous thromboembolism.

1996 ◽  
Vol 76 (05) ◽  
pp. 689-691 ◽  
Author(s):  
M A Crowther ◽  
M Johnston ◽  
J Weitz ◽  
J S Ginsberg

SummaryIn order to determine if there is a relationship between antiphospholipid antibodies and reduced free protein S levels, we evaluated 21 patients who had an antiphospholipid antibody but had neither a history of venous thromboembolism nor systemic lupus erythematosus (cases) and 55 matched controls, who did not have an antiphospholipid antibody, a history of thrombosis or systemic lupus erythematosus. Cases and controls had similar protein C and antithrombin levels. Six of 21 cases had reduced free protein S antigen levels, compared to 5 of 55 controls (x 2 = 5.823 p <0.025). In addition, the mean free protein S level was significantly lower in cases than in controls (0.30 ± 0.09 units vs 0.39 ± 0.13 units, p <0.01, two-tailed Student’s t-test). We conclude that antiphospholipid antibodies are associated with a significant decrease in free protein S levels, and that this acquired free protein S deficiency may contribute to the thrombotic diathesis seen in patients with antiphospholipid antibodies.


1989 ◽  
Vol 61 (01) ◽  
pp. 144-147 ◽  
Author(s):  
A Girolami ◽  
P Simioni ◽  
A R Lazzaro ◽  
I Cordiano

SummaryDeficiency of protein S has been associated with an increased risk of thrombotic disease as already shown for protein C deficiency. Deficiencies of any of these two proteins predispose to venous thrombosis but have been only rarely associated with arterial thrombosis.In this study we describe a case of severe cerebral arterial thrombosis in a 44-year old woman with protein S deficiency. The defect was characterized by moderately reduced levels of total and markedly reduced levels of free protein S. C4b-bp level was normal. Protein C, AT III and routine coagulation tests were within the normal limits.In her family two other members showed the same defect. All the affected members had venous thrombotic manifestations, two of them at a relatively young age. No other risk factors for thrombotic episodes were present in the family members. The patient reported was treated with ASA and dipyridamole and so far there were no relapses.


1989 ◽  
Vol 62 (03) ◽  
pp. 1040-1040 ◽  
Author(s):  
P Sié ◽  
B Boneu ◽  
R Biermé ◽  
M L Wiesel ◽  
L Grunebaum ◽  
...  

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