[Skin diseases due to systemic vasculitides and vasculopathies]

Vasculitis and vasculopathy are two distinct disease entities. Each entity comprises a large number of heterogeneous diseases, which can occur alone or associated with autoimmune, infectious or neoplastic diseases. The terms vasculitis and vasculopathy are often falsely used synonymously. A vasculitis initially causes inflammation of the vessel walls that may result in a secondary occlusion. In contrast, a vasculopathy is a primary occlusion of the vascular lumen, which is followed by inflammation after ischemia and ulceration. In most patients the distinction can be made based on the clinical presentation. A clear clinical diagnosis is then followed by targeted serological, histological and imaging procedures to confirm the clinical diagnosis. On this basis a well-founded treatment can be initiated. In the presence of vasculitis an anti-inflammatory therapy is indicated, whereas in the case of vasculopathy, removal of the vascular occlusion is the main focus. This article provides an overview of the various diseases and addresses the pathogenetic and clinical characteristics used to differentiate the individual disease entities. It also provides an insight into the therapy options and prophylaxis.