This Article Full Text Full Text (PDF) All Versions of this Article: 35/6/1329    most recent 01.STR.0000127534.54538.15v1 Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Ayoub, N. Articles by Francès, C. Search for Related Content PubMed PubMed Citation Articles by Ayoub, N. Articles by Francès, C. Related Collections Arterial thrombosis Thrombosis risk factors

(Stroke. 2004;35:1329.)
© 2004 American Heart Association, Inc.

Original Contributions

Protein Z Deficiency in Antiphospholipid-Negative Sneddon’s Syndrome

Nakhlé Ayoub, MD; Gaetan Esposito, Dr; Stéphane Barete, MD; Claudine Soria, PhD; Jean-Charles Piette, MD Camille Francès, MD

From Internal Medicine-Dermatology Department (N.A., S.B., J.-C.P., C.F.), Pitié-Salpêtrière Hospital, Paris; and Haematology Department (G.E., C.S.), Lariboisière Hospital, Paris, France.

Correspondence Prof Camille Francès, Internal Medicine Department, Pitié Hospital, 43-87 Boulevard de l’Hôpital, 75013 Paris. E-mail camille.frances{at}psl.ap-hop-paris.fr

Background— Sneddon’s syndrome is characterized by the association of ischemic cerebrovascular events and widespread livedo racemosa. The pathophysiology of Sneddon’s syndrome remains elusive, but various prothrombotic abnormalities have been previously reported in this setting. Low levels of protein Z, a downregulator of coagulation, have been recently linked to an increased risk of arterial thrombosis. The purpose of this study was to investigate the levels of protein Z in a series of Sneddon’s syndrome patients without circulating antiphospholipid antibodies in comparison with an age- and sex-matched control population.

Methods— Twenty-six patients and 78 healthy controls had determination of their protein Z blood levels by an enzyme-linked immunoassay test. Patients’ thrombotic and vascular risk factors, including tobacco smoking, arterial hypertension, oral contraceptive agents, dyslipidemia, factor V Leiden, and factor II mutation were recorded.

Results— Protein Z plasma levels were significantly lower in patients (mean 1.47 mg/L) than in controls (mean 1.93 mg/L) (P=0.02). Prevalence of protein Z deficiency (level <1 mg/L) was significantly higher (P=0.001) among patients (31%) than among controls (3.8%). Factor V Leiden and heavy smoking were observed in 4 and 7 patients, respectively.

Conclusions— Sneddon’s syndrome could be viewed as the peculiar clinical expression of various and sometimes associated coagulation abnormalities. Low levels of protein Z may account, at least partly, for the thrombotic events observed in Sneddon’s syndrome and shed a new light on its pathophysiology. Clinical implications for protein Z deficiency in this setting deserve further investigations.

Key Words: protein Z • Sneddon’s syndrome • livedo racemosa

This article has been cited by other articles:

				M. J. Heeb, K. M. Cabral, and L. Ruan Down-regulation of Factor IXa in the Factor Xase Complex by Protein Z-dependent Protease Inhibitor J. Biol. Chem., October 7, 2005; 280(40): 33819 - 33825. [Abstract] [Full Text] [PDF]

				J. Staton, M. Sayer, G.J. Hankey, V. Cole, J. Thom, and J.W. Eikelboom Protein Z Gene Polymorphisms, Protein Z Concentrations, and Ischemic Stroke Stroke, June 1, 2005; 36(6): 1123 - 1127. [Abstract] [Full Text] [PDF]