Clinicopathological Cases

TL;DRAbstract

We present the case of a 57-year-old woman with antineutrophil cytoplasmic antibody (ANCA)-negative systemic vasculitis diagnosed in December 2004 characterized by urticarial vasculitis, arthritis and livedo reticularis. She was poorly controlled on prednisolone, tacrolimus and hydroxychloroquine, having previously failed to be controlled on mycophenolate mofetil, methotrexate and cyclophosphamide. She presented acutely with a severe flare of vasculitis with myalgia, arthralgia and a flare of her urticarial vasculitis. The vasculitis was not controlled following pulsed methylprednisolone therapy. The skin eruption evolved rapidly into painful haemorrhagic bullae over the acral sites with associated abdominal pain. The pattern of urticarial vasculitis lesions dramatically changed, with large nonscaly concentric erythematous coalescing rings over her lower legs surrounded by a purple halo. Given the erythema gyratum repens-like pattern, a computed tomography scan of chest, abdomen and pe

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