It is important in the differential diagnosis of vasculitis to be aware of disorders that may present with livedo or infracted lesions secondary to occlusion disorders. These include cryo-globulinemic vasculitis, cholesterol embolization, Sneddon's disease, and malignant atrophic papulosis (Degos' disease). The major pathology in these is either initially occlusive or mediated by antiphospholipid antibodies and therefore falls into the category of microvascular occlusion.
Given the wide array of systemic diseases which can be associated with CSVV, it is important to carefully evaluate each patient for coexistent disease; keeping in mind that the first manifestation of large vessel vasculitis is often small vessel disease.
Screening laboratory abnormalities associated with autoimmune diseases in the work up for CSVV
Direct immunofluorescence microscopy Complement level
Anticardiolipin antibody lupus anticoagulant
Hypocomplementemic urticarial vasculitis
Antiphospholipid antibody syndrome
Antineutrophil cytoplasm antibodies
Decreased C1, C4, C2
Decreased C1q, C4, C2, C3, factor B and
Anti-C1q precipitin and/or decreased C1
IgG and/or IgM (measured by a standardized ELISA) for b2-glycoprotein I-dependent anticardiolipin antibodies and/or lupus anticoagulant activity the antibodies and/or activity should be found on two or more occasions, at least 6 weeks apart c-ANCA
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