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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 “acute hemorrhagic edema of infancy” and how does it differ from Henoch-Schönlein purpura?

Acute hemorrhagic edema of a young child demonstrating hemorrhagic indurated plaques of the head. As seen here, the ears and cheeks are frequently involved. (Courtesy of the Joanna Burch Collection.)
Fig. 15.2 Acute hemorrhagic edema of a young child demonstrating hemorrhagic indurated plaques of the head. As seen here, the ears and cheeks are frequently involved. (Courtesy of the Joanna Burch Collection.)
Acute hemorrhagic edema of infancy, which also called “cockade purpura,” is also an IgA-mediated immune complex leukocytoclastic vasculitis that affects small cutaneous vessels. This variant almost always affects young children (median age is 11 months), and like Henoch-Schönlein purpura, it is usually associated with a preexisting upper respiratory infection. It differs in that the primary cutaneous clinical lesions are typically indurated, edematous plaques with variable hemorrhage that are less likely to ulcerate. The lesions frequently affect the head and neck region and extremities (Fig. 15-2). The children typically do not demonstrate significant involvement of the gastrointestinal tract, joints or kidneys and do not develop permanent sequelae.

Fiore E, Rizzi M, Ragazzi M, et al: Acute hemorrhagic edema of young children (cockade purpura and edema): a case series and systematic review, J Am Acad Dermatol 59:684–695, 2008.