Urticaria Vasculitis
Urticaria vasculitis (UV) is a clinicopathological entity characterized by urticarial papules and plaques lasting longer than 24 h accompanied by histopathologic features of leukocytoclastic vasculitis. Clinical presentation of UV is characterized by urti
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5.9
Lluís Puig
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Urticaria vasculitis (UV) is a clinicopathological entity characterized by urticarial papules and plaques lasting longer than 24 h accompanied by histopathologic features of leukocytoclastic vasculitis. Clinical presentation of UV is characterized by urticarial papules or plaques, which can be burning or tender rather than pruritic in 33% of patients, usually persist for more than 24 h leaving residual purpura or hyperpigmentation, and are frequently associated with angioedema. The minimal histopathological criteria for UV have been proposed to include the presence of leukocytoclasis or fibrinoid necrosis in the lesions, irrespective of red blood cell extravasation. A continuum exists in the histopathologic changes of UV, which might reflect the time of evolution of lesions at the moment of biopsy. UV is considered to be due to deposition of immune complexes, which can be found in the circulation of 30–75% of patients. Patients with UV and complement activation are much more likely to have systemic disease than are patients with normocomplementemic UV. As many as 50% have moderate to severe emphysematous chronic obstructive pulmonary disease, and up to 30% have ocular inflammation, particularly uveitis. UV has an unpredictable course, even though the outcome is usually benign in patients with the idiopathic, normocomplementemic form of the disease.
L. Puig Department of Dermatology, Hospital Santa Creu i Sant Pau., Universitat Autònoma de Barcelona, Sant Antoni Maria Claret 167, 08025 Barcelona, Spain e-mail: [email protected] T. Zuberbier et al. (eds.), Urticaria and Angioedema, DOI: 10.1007/978-3-540-79048-8_5.9, © Springer Verlag Berlin Heidelberg 2010
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5.9.1 Definition Urticaria vasculitis (UV) is a clinicopathological entity characterized by urticarial papules and plaques of longer than 24 h duration – typically resolving with faint purpura or residual hyperpigmentation, indicating red blood cell extravasation – accompanied by histopathologic features of leukocytoclastic vasculitis in the capillaries and postcapillary venules of the skin. There should be evidence of leukocytoclasis and vessel wall destruction, which may or may not be accompanied by fibrinoid deposits.
5.9.2 Epidemiology UV is relatively uncommon; in patients presenting with chronic urticaria, its prevalence is 5% [1–3], with women accounting for 60% [4] to 80% [5] of cases and a peak incidence in the fourth decade of life [4, 5].
5.9.3 Histopathologic Findings The histopathology of UV often reveals most features of leukocytoclastic vasculitis, including endothelial cell injury and swelling, with disruption or occlusion of the vessel wall; extravasation of red blood cells, leukocytoclasis, fibrin deposition in and around the blood vessels; and a perivascular infiltrate composed mostly of neutrophils, even though lymphocytes can predominate in older lesions. Fibrinoid change or necrosis and particularly thrombosis are less common in UV than in fully developed lesions of palpable pu
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