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Livedo racemosa and digital necrosis in a patient with primary seronegative antiphospholipid syndrome and fibromuscular dysplasia of peripheral arteries

given as a maintenance therapy. The clinical remission lasted for two further months. In November 2005, a mild relapse of cutaneous lesions, mainly localized on the abdomen, was controlled by intravenous rituximab 375 mg m (four infusions at 2-monthly intervals) until June 2006. In September 2006 the patient was free of symptoms and the anti-BP180 antibody level was 27.3 IU mL (Fig. 2). This condition remained unchanged at last follow-up in December 2006, and maintenance therapy consisted of prednisone 12.5 mg daily and dapsone 75 mg daily. No side-effects of rituximab were observed. Rituximab is a chimeric monoclonal antibody directed against the CD20 receptor located on pre-B, naive and memory B lymphocytes. In the last few years rituximab has been used to treat various refractory autoantibody-mediated autoimmune diseases. Several case reports and our personal experience suggest that rituximab can induce long-term remission in patients with autoimmune bullous cutaneous diseases, including pemphigus vulgaris. In these patients significant B-cell depletion is associated with clinical improvement, although it is not always accompanied by a decline in the autoantibody titres. In our patient, the levels of anti-BP180 antibodies measured by ELISA showed a good correlation with the disease activity. In conclusion, rituximab seemed to be an effective and well-tolerated treatment for this patient with severe persistent PG. Further study is warranted to elucidate the role of this drug in the treatment of persistent PG and to define its safety profile in this group of patients.