[Systemic autoimmune diseases with neurocutaneous alterations]

Autoimmune diseases depend on the pathologic activation of cellular clones, potentially harmful, against soft tissues. They can be classified into systemic and organ-specific diseases. In the systemic subtype, in which the abnormal hyperactivity is not as specific, the most remarkable component of the clinical syndrome is frequently the presence of neurological and/or cutaneous manifestations. This article reviews the major pathogenic mechanisms involved in the principal systemic autoimmune conditions including connective tissue diseases (rheumatoid arthritis, systemic lupus erythematosus, polimyositis, dermatomyositis, scleroderma, mixed connective tissue disease, Sjögren's syndrome, overlap syndromes) and vasculitic syndromes (nodous polyarteritis, giant cell arteritis. Wegener's granulomatosis. Behçet disease, microscopic polyarteritis, cryoglobulinemia) as well as their clinical manifestations, focused mainly on neurocutaneous characteristics.