Localized Forms of Vasculitis

Abstract

Purpose of review: To provide an updated review on epidemiology, clinical manifestations, diagnostic assessment, treatment, and prognosis of localized vasculitis, following the 2012 Revised International Chapel Hill Consensus Conference Nomenclature on single-organ vasculitis.

Recent findings: Localized, single-organ vasculitides encompass a group of rare conditions in which there is no evidence of concomitant systemic vasculitis. Most data on this topic derives from case reports and small case series. Although some aspects of these diseases, such as clinical manifestations and histologic findings, have already been extensively investigated, there is still a lack of robust data concerning the pathogenesis, epidemiology, and treatment. Localized vasculitides may have a wide range of clinical features depending on the organ affected. The inflammatory process may have a multifocal/diffuse or unifocal distribution. Diagnosis is usually based on histopathology findings and exclusion of systemic vasculitis, which may frequently pose a challenge. Further research on treatment is warranted.

Keywords: Cutaneous arteritis; Cutaneous leukocytoclastic angiitis; Isolated aortitis; Single-organ vasculitis.

Fig. 1

Cutaneous leukocytoclastic angiitis. (a) Patient with CLA in lower extremities. (b) Inflammation in superficial dermis mostly involving small venules (black arrows), with predominant number of neutrophils and extravasation of erythrocytes (red arrow) (H&E × 25). (c) Infiltration of vessel walls with neutrophils which also extend into de perivascular zone and beyond (black arrows) (H&E × 100). (d) Visible vessel wall damage with necrosis, hyalinization and fibrin, and inflammatory cells in vessel wall (H&E × 400)

Fig. 2

Proposed treatment algorithm for cutaneous leukocytoclastic angiitis. (1) If refractory primary CLA, urticarial vasculitis, or IgA vasculitis. (2) If CLA secondary to rheumatoid arthritis. (3) If urticarial vasculitis

Fig. 3

Cutaneous arteritis. (a) Affected artery in the upper subcutis showing marked fibrin extravasation into the wall (black arrow) (H&E × 100). Inflammation is localized to the vessel and its immediate vicinity. (b) The fragmentation of the internal elastic lamina (arrow) is best appreciated by the use of a stain for elastic tissue (orcein × 200)

Fig. 4

Proposed treatment algorithm for cutaneous arteritis. (1) Severe disease: presence of ulcers, necrosis, or neuropathy. (2) Infliximab or etanercept (evidence based on pediatric cases)

Fig. 5

Computed tomography angiogram of a patient with isolated aortitis. Axial view (a) and sagittal view (b) showing concentric wall thickening of the aortic arch and descending thoracic aorta (green arrows)