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International Journal of Rheumatology
Volume 2010 (2010), Article ID 747946, 8 pages
http://dx.doi.org/10.1155/2010/747946
Clinical Study

Lower Extremity Ulcers in Systemic Sclerosis: Features and Response to Therapy

1Division of Rheumatology, Immunology and Allergy, Georgetown University Hospital, 3800 Reservoir Road, N.W., Hington, DC 20007, USA
2Centre for Biomedical Sciences, Cardiff School of Health Sciences, University of Wales Institute-Cardiff, Western Avenue, Cardiff CF5 2YB, UK
3Center for Wound Healing, Georgetown University Hospital, Washington, DC 20007, USA

Received 15 April 2010; Revised 15 June 2010; Accepted 2 July 2010

Academic Editor: Laura K. Hummers

Copyright © 2010 Victoria K. Shanmugam et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Nondigital lower extremity ulcers are a difficult to treat complication of scleroderma, and a significant cause of morbidity. The purpose of this study was to evaluate the prevalence of nondigital lower extremity ulcers in scleroderma and describe the associations with autoantibodies and genetic prothrombotic states. A cohort of 249 consecutive scleroderma patients seen in the Georgetown University Hosptial Division of Rheumatology was evaluated, 10 of whom had active ulcers, giving a prevalence of 4.0%. Patients with diffuse scleroderma had shorter disease duration at the time of ulcer development (mean 4.05 years 0.05) compared to those with limited disease (mean 22.83 years 5.612, P value .0078). Ulcers were bilateral in 70%. In the 10 patients with ulcers, antiphospholipid antibodies were positive in 50%, and genetic prothrombotic screen was positive in 70% which is higher than expected based on prevalence reports from the general scleroderma population. Of patients with biopsy specimens available , fibrin occlusive vasculopathy was seen in 100%, and all of these patients had either positive antiphospholipid antibody screen, or positive genetic prothrombotic profile. We recommend screening scleroderma patients with lower extremity ulcers for the presence of anti-phospholipid antibodies and genetic prothrombotic states.