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Vasculitis

»How are vasculitic disorders defined and classified?
»Are there specific serologic markers for any of these vasculitic disorders?
»What is a leukocytoclastic vasculitis?
»What are some important precipitating causes of small vessel leukocytoclastic vasculitis?
»What is Henoch-Schönlein purpura?
»What is the mnemonic that can help remember the clinical features of Henoch-Schönlein purpura?
»What is “acute hemorrhagic edema of infancy” and how does it differ from Henoch-Schönlein purpura?
»What are cryoglobulins?
»Can cryoglobulins produce a vasculitis?
»What is Churg-Strauss syndrome?
»What were those features again?
»What is Wegener’s granulomatosis?
»What are the features needed to establish a diagnosis of Wegener’s granulomatosis.
»Is there an easy way to remember these diagnostic criteria?
»List the cutaneous findings in Wegener’s granulomatosis.
»Wegener’s granulomatosis and Churg-Strauss syndrome seem very similar. How do you distinguish between them?
»What forms of treatment are available for Wegener’s granulomatosis and Churg-Strauss syndrome?
»What are the major organs involved in classic (systemic) polyarteritis nodosa (PAN)?
»How is classic polyarteritis nodosa different from Kawasaki disease?
»What is primary cutaneous polyarteritis nodosa?
»What is the primary difference between microscopic polyangiitis and PAN?
»What is the difference between giant cell (temporal) arteritis and Takayasu arteritis?
»What is erythema elevatum diutinum?
»Are there any other obscure disorders known to dermatologists, but little known to other subspecialties, that could be classified as vasculitis?

 
 
 

Are there specific serologic markers for any of these vasculitic disorders?

Yes. Antimyeloperoxidase antibodies directed against cytoplasmic components of neutrophils have been used to help identify patients with segmental necrotizing glomerulonephritis and some types of systemic vasculitis. Antibodies directed against serine proteinase 3 that is found in the cytoplasm of neutrophils (c-ANCA) have been detected in 66% to 90% of patients with active Wegener’s granulomatosis. Patients with pulmonary-renal syndrome who have antibodies directed against cytoplasmic myeloperoxidase, in neutrophils that produce a peripheral antineutrophil cytoplasmic pattern (p-ANCA), are most likely to have microscopic polyangiitis.

Harper L, Savage CO: Pathogenesis of ANCA-associated systemic vasculitis, J Pathol 190:349–359, 2000.