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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 erythema elevatum diutinum?


Erythema elevatum diutinum. Violaceous painful plaques on the dorsum of the hands. (Courtesy of the Fitzsimons Army Medical Center teaching files.)
Fig. 15.5 Erythema elevatum diutinum. Violaceous painful plaques on the dorsum of the hands. (Courtesy of the Fitzsimons Army Medical Center teaching files.)
Erythema elevatum diutinum is a rare form of small vessel vasculitis that is confined to the skin. This chronic disorder is characterized by persistent, elevated, erythematous plaques and/or nodules, with a predilection for overlying joint spaces, such as the fingers, wrists, elbows, knees, ankles, and toes (Fig. 15-5). The lesions are usually painful. Biopsies of developed lesions demonstrate a chronic leukocytoclastic vasculitis with extensive tissue fibrosis. There may be an associated IgA or, less commonly, an IgG monoclonal gammopathy (immune complex disease) and an association with inflammatory bowel disease, rheumatoid arthritis, systemic lupus erythematosus, streptococcal infection, IgA monoclonal gammopathy, multiple myeloma, myelodysplasia, celiac disease, relapsing polychondritis, and human immunodeficiency virus (HIV) infection. Some patients have shown a dramatic response to dapsone.

Farley-Loftus R, Dadlani C, Wang N, et al: Erythema elevatum diutinum, Dermatol Online J 14(10):13, 2008.