A 37-year-old woman with a 13-year history of widespread livedo reticularis and recurrent, painful ulcerative skin lesions was referred to our department because of a relapse of cutaneous manifestations of the skin lesions involving almost the whole body surface; malar erythema and oedema, non-scarring alopecia and fever were also associated. Routine laboratory data, immunological investigations and coagulation parameters were normal or negative. Histology was consistent with livedoid vasculopathy. A good clinical response was obtained using intravenous methylprednisolone combined with pentoxifylline. Livedoid vasculopathy is a rare, distinctive dermatosis that can be associated with systemic autoimmune disorders or present in an "idiopathic" form. The latter is at present regarded as a non-inflammatory thrombotic disease that may occur in patients with coagulation abnormalities. It is noteworthy that, in the present case, despite long-standing and dramatic cutaneous features, serious systemic complications have not developed and the patient's seroimmunologic and coagulative profile has remained normal.