TITLE:
Lower Extremity Ulcers in Patients with Systemic Sclerosis
AUTHORS:
Marta B. Bean, Chris T. Derk
KEYWORDS:
Lower Extremity Ulcers, Systemic Sclerosis, Scleroderma, Peripheral Vascular Disease, Venous Stasis
JOURNAL NAME:
Open Journal of Rheumatology and Autoimmune Diseases,
Vol.12 No.1,
February
25,
2022
ABSTRACT: Introduction: Cutaneous manifestations of systemic sclerosis (SSc) include skin
ulceration; 4% - 12% of patients with SSc develop lower extremity ulcers of
various etiologies. Limited data, significant morbidity, and substantial cost
of wound care led us to undertake this study to describe and identify risk
factors. Methods: After Institutional Review Board approval, we
identified 30 patients with SSc and lower extremity ulcers over a 10-year
period at a single center with an SSc clinic, which were included in a
descriptive analysis. Results:
Median age of onset of lower extremity ulcers was 59.5 years (range 20 - 84).
Ninety percent of patients were female, 60% were Caucasian, 63% had limited
SSc, 13% diffuse SSc and 23% an overlap syndrome. Immunomodulators or steroids
were prescribed in 53%; hypercoagulable state identified in 16%. Ulcers were
attributed to venous stasis (27%), SSc (20%), trauma (20%), arterial disease
(17%), and multifactorial/unknown (17%). In patients with ulcers attributed to
SSc, age at onset was lower (45.5 vs 59.5 years). Biopsies generally did not
contribute to management. Multidisciplinary treatment was routine; 20% required
amputation, 10% endovascular intervention, 20% frequent surgical debridement,
10% hyperbaric oxygen, 26% local treatment and antibiotics and 13% received
immunosuppression for wound treatment. Conclusion: Lower extremity ulcers are a serious clinical problem in
patients with SSc. The clinical exam, venous dopplers, ankle-brachial indices
and assessment of vascular risk factors helped define causality. In younger
patients, ulcers were more frequently attributed to SSc and these patients were
more likely to be on immunosuppressants/DMARDS, possibly indicating severe
phenotype of SSc.