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Tamihiro Kawakami, MD, PhD; Masahide Yamazaki, MD, PhD; Masako Mizoguchi, MD, PhD; Yoshinao Soma, MD, PhD

Arch Dermatol. 2009;145(2):171-175.

Background  Superficial vein thrombophlebitis is the common vascular symptom in Behçet disease and is characterized as erythema nodosum–like eruptions. Some studies have reported the presence of antiphospholipid antibodies (Abs) in patients with Behçet disease.

Observations  We measured lupus anticoagulant, anticardiolipin, anti–β₂-glycoprotein I, and antiphosphatidylserine-prothrombin complex antibody (Ab) levels in 3 patients with Behçet disease involving superficial vein thrombophlebitis. High levels of IgM antiphosphatidylserine-prothrombin complex Abs were found (mean [SD], 50.3 [43.1] U/mL; normal, <10 U/mL). One of the patients with Behçet disease was positive for both IgM and IgG antiphosphatidylserine-prothrombin complex Abs, and 2 were positive for lupus anticoagulant. Two patients were also positive for IgM anticardiolipin Abs, but the titers were low. In contrast, none of the patients with Behçet disease was positive for IgG anticardiolipin Abs or IgG or IgM anti–β₂-glycoprotein I Abs.

Conclusions  A high titer of IgM antiphosphatidylserine-prothrombin complex Abs was found in our patients with Behçet disease involving superficial vein thrombophlebitis. We speculate that there is a relationship between the antiphospholipid Abs, especially IgM antiphosphatidylserine-prothrombin complex Abs, and superficial vein thrombophlebitis complications in Behçet disease. This study suggests that elevated serum antiphosphatidylserine-prothrombin complex Ab levels might play some role in the development of the vascular manifestations in Behçet disease.

Author Affiliations: Department of Dermatology, St Marianna University School of Medicine, Kawasaki, Japan (Drs Kawakami, Mizoguchi, and Soma); and Department of Cellular Transplantation Biology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan (Dr Yamazaki).

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