ReviewSkin involvement in cutaneous and systemic vasculitis
Introduction
Cutaneous vasculitis is defined as a pathological process characterized by inflammation of the skin blood vessel wall; the disease process causes an alteration of the blood flow, ischemia, and damage to the neighboring tissue [1]. Although any type of vessel can be affected, the skin veins, particularly post-capillary venules, are more often involved than arteries and capillaries. In fact, post-capillary venules are mainly affected in cutaneous small vessel vasculitis (CSVV), also known with the histologic term of leukocytoclastic vasculitis, which is the most common form of vasculitis in dermatology [2]. Cutaneous vasculitides may be triggered by various antigenic agents, such as infection or drug, or may be related to an underlying disease, such as connective tissue, inflammatory bowel, myelodysplastic or other malignancies. However, it is important to note that many vasculitides occur without any demonstrable triggering agents, leading to consider the vasculitic process as “idiopathic”. On the other hand, vasculitic skin lesions may manifest as a component of vasculitis affecting also internal organs, cutaneous involvement being sometimes the initial sign of disease.
The classification of vasculitides has been a confusing and debate-provoking topic over the last half century. Despite numerous attempts, the development of a clinically relevant and easy-to use classification system that incorporates clinical features, vessel site, histopathological and laboratory findings, and possible etiologic factors, is a goal that has not yet been fully achieved. Thus, we will adopt a recently proposed working classification for vasculitides [3] which is useful for the scope of the present review (Table 1). Here, we will focus on the most frequent clinical variants of cutaneous vasculitis such as CSVV and urticarial vasculitis (UV) as well as the variety of cutaneous manifestations that may be observed in the course of the main systemic vasculitides such as polyarteritis nodosa (PAN), Wegener's granulomatosis (WG) and Churg–Strauss syndrome (CSS). Henoch–Schönlein purpura (HSP) will be argued among the cutaneous vasculitides based on that skin involvement is often predominant [1], [4], [5]. Unique forms of cutaneous vasculitis of specific interest for dermatologists, such as erythema elevatum diutinum, granuloma faciale or nodular vasculitis, will not be discussed.
Livedoid vasculopathy (LV), a cutaneous disorder which, albeit nontruly vasculitic in origin, is frequently associated with antiphospholipid antibody syndrome and/or connective tissue diseases [6], [7], will be discussed because may be of interest not only for dermatologists but also for immunologists and general readers.
Section snippets
Definition
Affecting mainly cutaneous post-capillary venules, cutaneous small vessel vasculitis (CSVV) is the most common type of vasculitis in dermatology. The cutaneous picture of CSVV is typically polymorphous and may include in particular palpable purpura, ulcers and urticarial lesions mainly involving the legs, while its extracutaneous manifestations are by definition relatively uncommon. It affects both children and adults, and is seen more commonly in women [8], [9], [10].
Etiologic factors
The main etiologic factors
Definition
Polyarteritis nodosa is a rare, severe necrotizing vasculitis of small- and medium-sized arteries affecting multiple organ systems. The disease has a segmental distribution and localizes chiefly in bifurcations and branchings of arteries [2]. Cutaneous manifestations of systemic PAN should be distinguished from those proper to the benign form confined to the skin [1].
Cutaneous features
Skin involvement can be found in about half of the patients with systemic PAN. The most common skin lesions are palpable purpura
Take-home messages
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Cutaneous vasculitis is a heterogeneous group of inflammatory disorders affecting skin blood vessels, particularly post-capillary venules.
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Cutaneous small vessel vasculitis (CSVV), also known as leukocytoclastic vasculitis, is the most common entity within this group.
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Many triggering factors may be identified in cutaneous vasculitis, but it is idiopathic in a relevant number of cases.
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The cutaneous picture of CSVV is polymorphous, including purpura, urticarial lesions and ulcers, while its
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