Differential diagnosis

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.

Table 7

Screening laboratory abnormalities associated with autoimmune diseases in the work up for CSVV

Laboratory test

Disease association


Direct immunofluorescence microscopy Complement level

Anticardiolipin antibody lupus anticoagulant

Collagen vascular disease Henoch-Schonlein purpura Rheumatoid arthritis Systemic lupus erythematosus

Hypocomplementemic urticarial vasculitis

Antiphospholipid antibody syndrome

Antineutrophil cytoplasm antibodies

Wegener's granulomatosis


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

Figure 4. Leukocytoclastic vasculitis. Low-power photomicrograph showing perivascular infiltrates and fibrinoid deposits within the vessels of the upper dermis. (Courtesy of Dr. Omar Sangueza, Wake Forest University School of Medicine, Winston-Salem, NC, USA.)

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