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

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 (n = 5), 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.

Vasculitic and autoimmune wounds

“…In both SSc and MCTD, end-organ damage occurs due to progressive tissue fibrosis and vasculopathy. Lower extremity ulcers are a known complication of longstanding scleroderma, affecting approximately 4% of patients and causing significant morbidity(21). In the previously described epidemiologic study of patients with chronic wounds, the prevalence of scleroderma in patients with chronic wounds was 2.35% compared to 0.02% in the general population (p<0.001)(6).…”

Vasculitic Diseases and Prothrombotic States Contributing to Delayed Healing in Chronic Wounds

“…Lower extremity ulcers are a known complication of longstanding scleroderma, affecting approximately 4% of patients and causing significant morbidity (20). The prevalence of scleroderma in patients with chronic wounds is 2.35% compared to 0.02% in the general population (p<0.001)(6).…”

“…The prevalence of scleroderma in patients with chronic wounds is 2.35% compared to 0.02% in the general population (p<0.001)(6). Histologically, fibrin occlusive vasculopathy with intimal thickening and some inflammation is seen (20). Scleroderma ulcers are bilateral in 70% of cases.…”

Successful Treatment with Bosentan of Lower Extremity Ulcers in a Scleroderma Patient

“…Bacterial superinfection occurred and many antibiotic treatments were necessary. Because of further deterioration intravenous administration of prostanoids in combination with subcutaneous administration of low molecular weight heparin [1] was started in May 2011. Only the ulcus on the left hallux responded partially (Figure 1(f)).…”

“…Lower extremity ulcers are less prevalent but are also a challenging and underestimated complication of the disease causing important pain and morbidity. In a recent clinical study the prevalence of nondigital ulcers in scleroderma patients was estimated at 4% [1]. …”