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May 2011

Severe Livedoid Vasculopathy Associated With Antiphosphatidylserine-Prothrombin Complex Antibody Successfully Treated With Warfarin

Author Affiliations

Author Affiliations: Department of Dermatology, University of Tokyo, Tokyo, Japan (Drs Noda, Asano, Ichimura, Tamaki, Takekoshi, Sugaya, and Sato); Cellular Transplantation Biology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan (Dr Yamazaki).

Arch Dermatol. 2011;147(5):621-623. doi:10.1001/archdermatol.2011.106

Livedoid vasculopathy (LV) is a chronic recurrent skin disease associated with various coagulopathies, clinically characterized by livedo reticularis, atrophie blanche, and painful ulcerations predominantly localized on the lower extremities. We herein report dramatic effects of warfarin therapy against rapidly progressive digital gangrene and foot ulcers in a patient with LV and antiphosphatidylserine-prothrombin complex antibody (aPS/PT).

A 66-year-old Japanese woman presented with a 40-year history of recurrent painful ulcerations on her lower extremities. Two weeks earlier, an ulceration involving her right dorsal foot and gangrene of the right toes emerged. Physical examination revealed gangrene of the right fourth and fifth toes and ulcerations on the right ankle and dorsal surfaces of both feet, and atrophie blanche (Figure 1A-C). A skin biopsy specimen showed nonspecific findings typically seen in chronic leg ulcers (Figure 2). While prothrombin time, activated partial thromboplastin time, and levels of proteins C and S, antinuclear antibody, antineutrophil cytoplasmic antibody, lupus anticoagulant, IgG and IgM anticardiolipin antibodies (aCLs), and β2 glycoprotein I–dependent anticardiolipin antibody were within normal ranges, IgM aPS/PT findings were strongly positive (60 U/mL; normal, <12 U/mL). Findings for IgG aPS/PT were also slightly positive (13 U/ml; normal, <12 U/mL).