Livedoid vasculopathy - further evidence for procoagulant pathogenesis

Objective: To further characterize the clinical and pathologic features, disease associations, and laboratory abnormalities of livedoid vasculopathy. Design: Retrospective study of patients identified from our institutional database from January 1, 1990, to December 31, 2000. Setting: Tertiary care institution. Patients: Forty-five patients with biopsy-proved livedoid vasculopathy. Main Outcome Measures: Clinical presentation, histopathologic diagnosis, results of testing for coagulation abnormalities, and assessment of vascular status. Results: Thirty-two patients (71.1%) were female (mean age, 45 years; age range, 10-85 years). Bilateral lower extremity disease occurred in 36 patients (80.0%), ulceration in 31 (68.9%), and atrophie blanche in 32 (71.1%). In patients tested, transcutaneous oximetry measurements were decreased in 20 (74.1%) of 27, and factor V Leiden mutation (heterozygous) was noted in 2 (22.2%) of 9, decreased activity for protein C or protein S in 2 (13.3%) of 15, prothrombin G20210A gene mutation in 1 (8.3%) of 12, and lupus anticoagulant in 5 (17.9%) of 28. Anticardiolipin antibodies were present in 8 (28.6%) of 28 patients, and elevated homocysteine levels in 3 (14.3%) of 21. Intraluminal thrombosis was observed in 44 (97.8%) of 45 skin biopsy specimens. Direct immunofluorescence disclosed multiple vascular conjugates in 31 (86.1%) of 36 biopsy specimens. Conclusions: Livedoid vasculopathy was predominantly bilateral, affected the lower extremities, and was associated with ulceration and atrophic blanche. Histologic evidence of intraluminal thrombosis was observed in almost all biopsy specimens reviewed. Laboratory testing revealed numerous heterogeneous coagulation abnormalities, providing further evidence of procoagulant mechanisms.