RT Book, Section A1 Merkel, Peter A. A1 Monach, Paul A. A2 Goldsmith, Lowell A. A2 Katz, Stephen I. A2 Gilchrest, Barbara A. A2 Paller, Amy S. A2 Leffell, David J. A2 Wolff, Klaus SR Print(0) ID 56078520 T1 Chapter 164. Systemic Necrotizing Arteritis T2 Fitzpatrick's Dermatology in General Medicine, 8e YR 2012 FD 2012 PB The McGraw-Hill Companies PP New York, NY SN 978-0-07-166904-7 LK accessmedicine.mhmedical.com/content.aspx?aid=56078520 RD 2024/03/29 AB |PrintSystemic Vasculitis at a GlanceHeterogenous group of rare inflammatory conditions in which blood vessel walls are primarily targeted by an inflammatory reaction.Etiology unknown; geographic, environmental, and genetic factors may be important.Incidence up to 42 cases per million per year.Diagnosis often established in patients with multiple but variable organ involvement; supported by laboratory or radiologic studies and histologic evidence of inflammatory reaction in blood vessels.Cutaneous involvement can occur in any of the primary systemic vasculitic syndromes.Cutaneous features are often not sufficient to provide complete diagnosis and staging.Classification of the vasculitides is based on several factors including known etiology or disease association (primary vs. secondary forms), predominant size of involved vessels (small, medium, or large arteries), and additional clinical and laboratory data.Primary vasculitis is a diagnosis of exclusion after causes of secondary vascular inflammation have been ruled out.Determinants of prognosis and treatment include the specific vasculitic syndrome, whether critical organs are involved, the severity of involvement, and the rate of disease progression.Treatment often requires glucocorticoids and the use of other immunosuppressive drugs.