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Giornale Italiano di Dermatologia e Venereologia 2004 October;139(5):389-413


language: English, Italian

Cutaneous small-vessel vasculitis

Ghersetich I., Buggiani G., Brazzini B., Lotti T.

Department of Dermatological Sciences University of Florence, Florence, Italy


Cutaneous small-vessels vasculitis (CSVV) is a clinical disorder comprising a large spectrum of cutaneous lesions, usually presenting as palpable purpura. The skin is often the only organ apparently involved, but clinically relevant systemic involvment may occur. In many cases the cause of CSVV remains unknown (idiopathic CSVV), in others CSVV has been reported in association with chronic diseases, malignant neoplasms and precipitating causes (drugs, chemicals, infections, food allergens). Histologically CSVV is characterized by angiocentric segmental inflammation, endothelial cells swelling and fibrinoid necrosis of blood vessels walls. Although blood vessels of any size may be affected in systemic vasculitis, CSVV occurs in the small venules (postcapillary venules), being characterized by 2 main histological patterns: a leukocytoclastic form in the first phase of the disease, with an immune complex mediated pathogenesis; and, lately, a lymphomonocytic form, due to the partecipation of a secondary cell mediated immune response in the late phase of the vasculitis. The cutaneous fibrinolytic activity is increased in the early phase and reduced in the late phase of the disease. g/d T lymphocytes and heat shock proteins are strongly represented only in lesional skin of cases of leukocytoclastic vasculitis with documented infective etiology, suggesting that the investigation of g/d T cells and heat shock proteins in CSVV might represent a clue to the infective etiology of CSVV. Therapeutic approach requires elimination of the cause (drugs, chemicals, infections, food allergens) when possible. In other cases, local and systemic antinflammatory or immunosoppressive therapies are recommended.

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