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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?

 
 
 

What is Churg-Strauss syndrome?

Churg-Strauss syndrome (allergic granulomatosis) is an uncommon multisystemic vasculitis that is characterized with asthma, eosinophilia, extravascular granulomas, and positive ANCA titers. The main systemic features of Churg-Strauss syndrome are summarized in Table 15-2. Pulmonary involvement and eosinophilia helps discriminate Churg-Strauss syndrome from polyarteritis nodosa. The primary cutaneous lesions most commonly consist of palpable purpura involving the extremities, although some patients may also demonstrate fixed papules or plaques or even subcutaneous nodules. Cutaneous lesions have been reported in 45% to 70% of patients. Cutaneous involvement should be considered an important feature when present; biopsies in addition to demonstrating vasculitis of small blood vessels, frequently demonstrate large numbers of eosinophils and, less commonly, may demonstrate extravascular granulomatous inflammation.
Table 15-2. Clinical Features of Churg-Strauss Syndrome
  FINDING SENSITIVITY
 
Asthma
  100%
 
Blood eosinophilia >10%
  95%
 
Paranasal sinus abnormalities
  86%
 
Mononeuropathy or polyneuropathy
  75%
 
Pulmonary infiltrates
  40%
 
Extravascular (perivascular) eosinophils
  14%
 
History of seasonal allergies
  -