Vasculitis

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Vasculitis is probably the most common nonspecific cutaneous lesion occurring in patients with SLE. Frequencies have varied from 20% to 70% (Dubois and Tuffanelli 1964, Estes and Christian 1971, Pistiner et al. 1991). Our experience with 150 patients with SLE reported a 27% frequency (Hochberg et al. 1985). Although the cutaneous manifestations of vasculitis can vary from urticarial-like lesions to deep ulcerations,

Table 7.1. Nonspecific cutaneous lesions in systemic lupus erythematosus

Vasculitis Palpable and nonpalpable purpura Urticarial-like vasculitis Hypocomplementemic urticarial vasculitis Livedo reticularis Photosensitivity Perniosis Alopecia

Raynaud's phenomenon Erythromelalgia Bullous lesions Arthritis

Sclerodermatous changes

Dermal mucinosis

Erythema multiforme-like lesions

Pigmentary changes

Nail changes

Cutis laxa

Lichen planus

Acanthosis nigricans

Angioedema the most common presentation is palpable and nonpalpable purpura of the lower extremities.

The intensity of the inflammatory response and its location in the vasculature of the skin determines the morphologic presentation. For example, vasculitic lesions involving arterioles and venules in the papillary portion of the dermis commonly present as nonpalpable purpura, whereas vasculitic lesions involving blood vessels in the middle portion of the reticular dermis present as palpable purpuric lesions. If the inflammatory insult is minor, the vasculitis may present as urticarial-like lesions, whereas if the vasculitic inflammation is intense, ulceration may occur.

Intense inflammatory responses involving blood vessels in the upper portion of the papillary dermis may result in destruction of the integrity of the dermal-epidermal junction, producing vesicles and bullae.

Vasculitic lesions involving the venules and arterioles at the tips of the fingers can produce small tender nodules (Osler nodes). Vasculitic lesions involving the thenar and hypothenar eminences and characterized by erythematous, generally nontender lesions have been referred to as Janeway spots. Inflammation of the arterioles in the cuticle nail folds can present as infarcts.

A putative immune complex process is thought to be causative. The reason for predominance of vasculitic lesions on the lower extremities or on the back in bedridden patients is probably due to hydrostatic pressure as well as local constriction of blood vessels producing eddying, facilitating immune complex deposition. Vasculitic lesions may occur at sites of trauma (e. g., following shaving of the legs) or at pressure sites such as around the waist. These sites of trauma or pressure application may also cause alteration in blood flow or the local release of histamine, promoting the deposition of immune complexes.

Biopsy specimens of most vasculitic lesions in SLE demonstrate the presence of a leukocytoclastic vasculitis. On infrequent occasions, a mononuclear vasculopathy is present. Direct immunofluorescence examination generally demonstrates the deposition of immunoglobulin, complement components, and fibrin in the affected blood vessels.

Although the weight of scientific data indicate that immune complexes are involved in the pathogenesis of the vasculitic lesions, patients with SLE can produce antibodies reactive against blood vessel endothelial cell surface antigens. Thus, from a conceptual point of view, it is conceivable that antibodies directed against these endothelial surface antigens could activate the complement sequence, inducing a prominent neutrophilic chemotactic response to the area of antigen antibody reaction, producing a similar, if not identical, histopathologic picture as leukocytoclastic vasculitis. It is also conceivable that antigen-committed T cells could also be involved in the pathogenesis of the vasculitis.

Urticaria as a manifestation of vasculitis in SLE has been increasingly recognized (O'Laughlin et al. 1978, Provost et al. 1980). At the present time, two forms of urti-carial-like vasculitis have been detected. The most common form, occurring in approximately 10% of patients with SLE, is associated with various autoantibodies (such as anti-double-stranded DNA, anti-Ro/SSA, anti-U1RNP antibodies). In general, these patients have clinical features of systemic disease, including glomeru-lonephritis and arthritis. Most patients demonstrate a leukocytoclastic angiitis, but, on a few occasions, a mononuclear vasculopathy has been described.

The second form of urticarial-like vasculitis seen in patients with SLE is characterized by the presence of anti-Clq antibodies and hypocomplementemia. This form of urticarial-like vasculitis (a leukocytoclastic angiitis) also can occur in patients without SLE and is termed "hypocomplementemic urticarial vasculitis (HUVS)" (Shu and Mannik 1988). Clinically, these patients frequently demonstrate arthralgias, arthritis, glomerulonephritis, angioedema, ocular inflammation (such as conjunctivitis, episcleritis, uveitis), and obstructive lung disease. Rarely, pericarditis is detected. Some patients with SLE have anti-Clq antibodies, and several patients initially presenting with HUVS subsequently develop SLE (Trendelenburg et al. 1999, Wisnieski and Jones 1992). These studies indicate that SLE and HUVS, once thought to be distinct entities, may be related, representing opposite ends of a spectrum of disease.

Clinically, the HUVS lesions and urticarial-like vasculitis are characterized by persistence, lasting 24 h or longer. The lesions, unlike those of classic urticaria (which are transient, lasting 6 h or less), are associated with a burning sensation rather than pruritus. The application of light touch to the individual lesions may produce hyper-pathia. On occasion, careful examination of the urticarial-like vasculitic lesion will demonstrate petechae, indicating disruption of the blood vessel integrity.

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