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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?

 
 
 

Wegener’s granulomatosis and Churg-Strauss syndrome seem very similar. How do you distinguish between them?

It is often difficult to distinguish between Wegener’s granulomatosis and Churg-Strauss syndrome due to the presence of nasal, sinus, and pulmonary involvement in both diseases and the fact that all the classic features are rarely found in a single patient. There are many situations in which the features of systemic vasculitides overlap, and these are referred to as overlap vasculitis syndromes, similar to the overlap syndromes reported for other rheumatologic conditions.

The use of cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) for differentiating between these two disorders is helpful (c-ANCA is usually present in high titers in Wegener’s but not in Churg-Strauss), but is not included in the current diagnostic criteria for either syndrome. The presence or absence of other features should confirm the diagnosis in most cases (Table 15-3).
Table 15-3 Wegener’s Granulomatosis versus Churg-Strauss Syndrome
      WEGENER’S   CHURG-STRAUSS
 
Asthma
  -   +
 
Blood eosinophilia
  -   +
 
Perivascular eosinophils on biopsy
  -   +
 
Hemoptysis
  +   -
 
Microhematuria
  +   -