Abstract
Livedoid vasculopathy is a chronic thrombotic disease of the skin microcirculation resulting in painful ulcers mainly affecting the lower legs. This study presents a French cohort of patients with livedoid vasculopathy. It describes clinical, histological characteristics and outcome of patients. It notably shows a frequent thrombophilia background. Nerve damage can be associated with cutaneous manifestations. These data also confirm that heparin or oral anticoagulants are able to achieve a complete response. Refractory cases can be treated with intravenous immunoglobulins. Livedoid vasculopathy is a rare thrombotic cutaneous disease. This observational study aimed to assess the clinical and biological features of livedoid vasculopa thy and the efficacy of treatments. Patients enrolled had typical livedoid vasculopathy both clinically and histologically. Investigation of thrombophilia was per formed. Electromyography was undertaken in the pre sence of symptoms suggesting peripheral neuro pathy. Eighteen women and 8 men were included, with a mean age of 35.5 years at onset. Twenty patients had at least one thrombophilia factor. Ten patients had a peripheral neuropathy with 2 of these patients demon strating a specific thrombo-occlusive vasculopathy on muscle biopsy. Anticoagulation with low molecular weight heparin was the most prescribed therapy and was associated with the best outcome (effective in 14 patients). Eight patients had severe disease refractory to anticoagulation and required intravenous immuno globulins, producing a good response in 6 patients.