Cutaneous involvement vascular lesions

Vascular lesions are quite frequent in pSS and their clinical manifestations are extremely various. Raynaud's phenomenon is probably the most common vascular feature seen in pSS with a prevalence varying from 15 to 35% (Provost and Watson, 1992; Bernacchi et al., 2004; Ramos-Casals et al., 2005). Moreover, it can be one of the earlier manifestations of the disease, preceding sicca symptoms by many years (Ramos-Casals et al., 2005). Unlike scleroderma patients, pSS patients with Raynaud's phenomenon present a mild vasospastic phenomenon and do not develop teleangiectasias or digital ulcers. Non-erosive arthritis has also been shown to be significantly more common in patients with Raynaud's phenomenon than in those without (Skopouli et al., 1990).

Cutaneous vasculitis includes a variety of lesions depending on the level of blood vessel involvement in the skin and the intensity of inflammatory response. The most common vasculitis lesions are flat and palpable purpura (Provost and Watson, 1992). Flat purpura is usually seen in patients with hype-rgammaglobulinemia, while palpable purpura is a manifestation of dermal vasculitis (Provost and Watson, 1992). Bloch et al. (1965) described purpura in 17% of their pSS patients. Pavlidis et al. (1982) found purpura, at disease onset, in 28% of the 47 patients described and Garcia-Carrasco et al. (2002) in 15% of their 253 patients analyzed. Finally, of the 62 pSS patients included in the study by Bernacchi et al. (2004), 19 presented with cutaneous vasculitis, while there were only nine in the group of 31 secondary Sjogren's syndrome patients. Palpable purpura was the most common clinical feature identified (Bernacchi et al., 2004). Purpura appears as recurrent crops of round, pink, separated or confluent lesions turning dull purple and brown in a few days and finally resolving or leaving a pale brown stain (Roguedas et al., 2004); in contrast with simple purpura, palpable purpura does not blanch when pressure is applied to the skin. Moreover, due to the increased hydrostatic pressure, it typically involves lower extremity and buttocks (Gonzalez-Gay et al., 2003). Cutaneous purpura has been associated with lymphoma development and mortality, cutaneous vasculitis thus becoming significant in the prognosis and outcome of patients with pSS (Voulgarelis et al., 1999; Ramos-Casals et al., 2005). Two different types of vasculits have been histopathologically described in pSS: the neutrophilic inflammatory vascular disease, indistinguishable from a leukocytoclastic vasculitis, and the mononuclear inflammatory vascular disease (Provost and Watson, 1992; Roguedas, et al., 2004). The first pattern is characterized by an inflammatory infiltrate composed predominantly by neutrophils, many of which are fragmented. Moreover, the lesions typically display fibrinoid necrosis, lumen occlusion and extravasation of red blood cells. The mononuclear inflammatory vascular disease is characterized by a mononuclear inflammatory infiltrate with invasion of the blood vessel walls. Fibrinoid necrosis is present, but less prominent than the neutrophilic inflammatory infiltrate (Provost and Watson, 1992; Roguedas et al., 2004). Despite the evidence of these two forms of vasculitis, their clinical expression is very similar, so that is not predictable, the histopathologic pattern of vascular insult, in examining the skin (Provost and Watson, 1992). Nonetheless, neutrophilic inflammatory vascular disease, in contrast with mononuclear inflammatory vascular disease is statistically associated with antinuclear antibodies, high titres of anti-Ro/SSA and anti-La/SSB antibodies, hypergammaglobulinemia, rheumatoid factor and hypocomplementemia (Oxholm et al., 1984; Provost and Watson, 1992 Roguedas et al., 2004).

The second most common form of inflammatory vascular disease is urticarial vasculitis, which is a manifestation of inflammatory injury of capillaries and postcapillary venules in the skin (Wisnieski,

2000). From a clinical point of view, urtical vasculitis differs from true urticaria for the following reasons: (i) urticarial vasculitis lesions can be pruritic, like in true urticaria, but are more likely to be stinging or burning; (ii) urticarial vasculitis lesions are typically 0.5-5 cm in diameter, while true urticaria may coalesce into larger lesions; (iii) ur-ticarial vasculitis lesions typically persist for 24 h and often resolve with hyperpigmentation, indicating red blood cell extravasation. In contrast, true urticaria persists for 2-8 h and leaves no trace (Wisnieski, 2000; Gonzalez-Gay et al., 2003). The histology of the urticarial vasculitis includes the features of a leukocytoclastic vasculitis with the neutrophilic infiltrate (or a mixed infiltrate of neu-trophils and lymphocytes), the extravasion of red blood cells and injury of the endothelial cells with disruption of the vessel wall. Fibrinoid change or necrosis are less common in urticarial vasculitis lesions than in the fully developed lesions of palpable purpura (Wisnieski, 2000).

In addition to these common manifestations of inflammatory vascular disease, pSS patients may rarely demonstrate erythematous nodules on the lower extremities, persistent plaque-like lesions (erythema multiforme-like lesions) as well as superficial ill-defined patches (erythema perstans) (Roguedas et al., 2004). Provost and Watson (1992) reported persistent plaque-like lesion in 9% of their patients with pSS and ill-defined superficial patches in 4% of them.

Sweet's syndrome, which is characterized by ery-thematosus -oedematous plaques and reflects inflammatory infiltration of the dermis with neu-trophils, has been occasionally reported in pSS (Vatan et al., 1997) In Japanese pSS patients annular erythema is often reported, while in Caucasian patients only anecdotic reports have been published (Katayama et al., 1991; Watanabe et al., 1997; Bernacchi et al., 2004). Annular erythema lesions have been described as recurrent, non photosensitive, erythematous-indurated plaque-like lesion with central clearing without pigmentation or atrophy. Lesions typically involve the face (especially the cheek and preauricular region), the upper extremities and the trunk and a correlation with anti-Ro/SSA and anti-La/SSB antibodies has been reported in as many as 78-100% of pSS cases

(Katayama et al., 1991; Watanabe et al., 1997). Histological examination revealed marked oedema of the upper dermis and a coat-sleeve-like infiltration of lymphocytes around the blood vessels throughout the dermis (Kawakami and Saito, 1999). Thus, Asian patients appear to have lesions that might be considered similar to Caucasian subacute cutaneous lupus erythematosus, although, the latter are not photosensitive and display a distinct histological profile (Watanabe et al., 1997; Bernacchi et al., 2004).

5.3.3. Cutaneous involvement: miscellaneous

A broad spectrum of other cutaneous manifestations has been described in pSS including angular cheilitis, eyelid dermatitis, vitiligo and alopecia (Bernacchi et al., 2004). Angular cheilitis has been described as recurrent, symmetrical erythemato-squamous infiltrated lesion, often itching and fis-suring and it is more common in primary than in secondary variants. Xerostomia may be a predisposing factor for the development of angular cheilitis (Bernacchi et al., 2004). Eyelid dermatitis is defined by the presence of erythematous, infiltrated and lichenificated lesions of the upper and/ or lower eyelids and it is associated with itching and foreign body sensation. It has been hypothesized that ocular dermatosis may not be a simple consequence of contact sensitization, but also the result of continuous rubbing of the periorbital area due to xerophthalmia (Bernacchi et al., 2004). Alopecia areata has also been described in pSS as well as in other autoimmune diseases, and it has been explained by a possible lymphocytic infiltration of the sebaceous glands (Provost and Watson, 1992). Moreover, vitiligo consisting in localized areas of hypopigmentation has been associated with pSS too (Roguedas et al., 2004). Finally, a true complication of pSS is represented by malignant lymphomas (Voulgarelis et al., 1999; Roguedas et al., 2004). Skin lymphomas are quite rare and data on their real incidence in pSS are lacking (Bernacchi et al., 2004). Nonetheless, many case reports have been published describing cutaneous B-cell lymphomas, or most infrequently T-cell lymphomas, and all these cases have to be considered as part of the increase risk of pSS proliferative disorders (Jubert et al., 1993; Voulgarelis et al., 1999; Stroehmann et al., 2002; Selva-O' Callaghan et al., 2003; Masaki and Sugai, 2004; Ramos-Casals et al., 2005a).

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