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Abbreviation ESR, erythrocyte sedimentation rate. Source: From Ref. 1.

Abbreviation ESR, erythrocyte sedimentation rate. Source: From Ref. 1.

picture that includes manifestations such as livedo reticularis, purpura, "purple toe syndrome," ulcer, infarct, and erythromelalgia. Biopsy of the skin allows separation of these vaso-occlusive disorders from vasculitis (Figs. 20-22).

Figure 20 shows a case of antiphospholipid antibody syndrome can present as cutaneous infarcts (Fig. 20A) that can mimic vasculitis. This syndrome is characterized by venous and arterial thromboses, fetal losses and thrombo-cytopenia, which are associated with the presence of anti-phospholipid antibodies. Histologically, noninflammatory vascular thrombi are identified (Figs. 20B and C) that affect arterioles, arteries, and veins. At most sparse, lymphocytic infiltrates will be found around the involved vessels associated with rare pyknotic endothelial nuclei. Incompetent or "leaky" blood vessels due to chronic inflammation, vessel wall pathology (e.g., amyloidosis), or dietary deficiencies (Scurvy—vitamin C deficiency) can lead to hemorrhage into the dermis that can be mistaken clinically for vasculitis. Solar or senile purpura, characterized by large ecchymoses over the extensor surfaces of forearms and hands of elderly individuals or in this case the forehead (Figs. 21A), is believed to be the result of minor injury to poorly supported vessels in the dermis as these patients typically have abundant solar elastotic material and atrophic dermal collagen bundles (Fig. 21B). Pigmented purpuric dermatoses are a group of chronic inflammatory, nonvasculitic dermatoses that all have in common a purpuric clinical appearance (Fig. 21C) and variable numbers of extravasated erythrocytes and hemosiderin deposits associated with perivascular lymphocytic inflammatory infiltrates without fibrinoid necrosis of vessels, histologically (Fig. 21D). Lastly, livedo vasculopathy (a.k.a., atrophie blanche, livedo vasculitis, and segmental hyalinizing vasculitis) is one relatively common disorder that can mimic, both clinically and histologically, vasculitis. It is a chronic thrombo-occlusive disorder of the feet and lower legs. Early lesions show petechiae, but characteristic features are recurrent, bizar-rely shaped ulcers that heal leaving hyperpigmentation and white atrophic stellate scars (atrophie blanche) (Figs. 22A and B). Most morphological studies have shown fibrin deposition within both the wall and lumen of affected vessels associated with ulcer, dermal fibrosis, and a perivascular lymphocytic infiltrates (Figs. 22B and C). If neutrophils are present, they are few and do not disrupt vessels or show leukocytoclasis, and are often sign of adjacent ulceration.

References:

1. Grau R. Pseudovasculitis: mechanisms of vascular injury and clinical spectrum. Curr Rheumatol Rep 2002; 4(1):83-89.

2. Sacks KE. Mimickers of vasculitis. In: Koopman WJ, Moreland LW, eds. Arthritis and Allied Conditions. 15th ed. Philadelphia: Lippincott Williams and Wilkins; 2005:1845-1867.

Figure 1 Cutaneous vasculitis classification is based on the size of vessels involved. Source: From Ref. 1.
Figure 2 Clinicopathologic correlation: cutaneous manifestations correlate with size of vessel affected by vasculitis. (Continued)

Figure 2

Continued.

Figure 2

Continued.

Figure 5 Direct immunofluorescence findings and pathophysiology of vasculitis. Source: C and D reproduced with permission from Savige JA et al. J Clin Pathol 1998; 51(8):568-575.
Figure 6 Cutaneous leukocytoclastic angiitis (leukocytoclastic vasculitis).
Figure 8 Urticarial vasculitis: two patients with urticarial vasculitis, both of whom showed perivascular nuclear debris (arrows) on biopsy are rare extravasated red blood cells.
Figure 9 Septic vasculitis due to Capnocytophaga canimorsus. Necrosis at the site of dog bite is a clinical clue to infection with Capnocytophaga canimorsus (B).
(A)
(B>
Figure 17 Nodular vasculitis due to tuberculosis (erythema induratum). Source: Clinical photo and histologic material courtesy of Harvey Lui MD and Nigel Ball MD, Vancouver, Canada.
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