Vasculitis and thrombotic thrombocytopenic purpura in a patient with limited scleroderma

Inflammatory vasculopathy and thrombotic thrombocytopenic purpura (TTP) are rare complications of scleroderma. We report a 54-year-old woman with limited cutaneous scleroderma who developed medium size and small vessel vasculitis. Inflammatory changes of medium size muscular arteries presented as ovarian vasculitis and mononeuritis multiplex, while arteriolar involvement presented as TTP with associated central nervous system involvement. In addition, possible noninflammatory involvement of small muscular arteries was expressed as Raynaud's phenomenon.