Systemic Disease

Thirty to 63% of SCLE patients have four or more American College of Rheumatology (ACR) diagnostic criteria for SLE (Sontheimer 1989; Chlebus et al. 1998; Parodi et al. 2000; Black et al. 2002). Musculoskeletal symptoms such as arthritis and arthralgias are the most common systemic manifestations observed. Overall, most patients with SCLE tend to have mild systemic disease and it appears that isolated joint symptoms are a marker for milder disease. Some authors have reported musculoskeletal symptoms in 100 percent of their SCLE cohorts (Molad et al. 1987; Johansson-Stephansson et al. 1989). Renal and central nervous system (CNS) disease has been seen in 20% or less of SCLE cohorts (Cohen and Crosby 1994; Sontheimer 1989; Johansson-Stephansson et al. 1989; Chlebus et al. 1998; Parodi et al. 2000, Black et al. 2002). SCLE cohorts who have nephritis, papulosquamous lesions, high ANAs (>1:640), or who require high dose immunosuppressive therapy may have a worse prognosis (Sontheimer 1985, Cohen and Crosby 1994, Tebbe et al. 1997, Chlebus et al. 1998). Fatalities have rarely been reported in patients with severe systemic manifestations (Sontheimer 1989; Gunmundsen et al. 1992).

Sjogren's syndrome is the most common autoimmune disease associated with SCLE. The HLA-B8, DR3, DRw6, DQ2, and DRw52 extended haplotype is common to both Sjogren's syndrome and SCLE cohorts (Provost et al. 1988). The association with HLA-DR is probably more related to high circulating Ro/SS-A autoantibodies rather than to SCLE skin lesions. High Ro/SS-A antibody titers have also been associated in Sjogren's and LE patients with HLA-DQw1/DQw2 (Harley et al. 1986; Hamilton et al. 1988). (Fig. 2). In early studies twelve percent of SCLE cohorts developed Sjogren's syndrome. (Sontheimer et al. 1981). Subsequent studies with longer observation periods have reported the coincidence of Sjogren's syndrome to be as high as 43% (Black et al. 2002). In SCLE patients, the presentation of Sjogren's syndrome may be atypical. Rapidly progressive hypokalemic flaccid tetraparesis caused by a distal renal tubular acidosis was attributed to unrecognized Sjogren's syndrome in an SCLE patient (De Silva et al. 2001). Furthermore, annular erythema of Sjogren's syndrome is considered to be the Asian counterpart of SCLE in white persons. These patients have annular lesions similar to SCLE; however, they lack histopathologic findings at the dermal-epidermal junction of LE. It has been suggested that this is a subset of SCLE and that the relative absence of HLA DR3 in Japanese patients may account for the differences in disease expression (Haimowitz et al. 2000).

Other autoimmune disorders associated with SCLE include rheumatoid arthritis (Cohen et al. 1986; Sontheimer 1989, Pantoja et al. 2002), autoimmune thyroiditis (Sontheimer 1989; Ilan and Ben Yahuda 1991), hereditary angioedema (Gudat and Bork 1989), and autoimmune polyglandular syndrome type II (Schmidt's syndrome) (Wollina and Schreiber, 2003).

SCLE has been associated with various malignancies and some authors have suggested that SCLE is a paraneoplastic dermatosis (Brenner et al. 1997). Reported malignancies include lung, gastric, breast, uterine and hepatocel-lular carcinoma (Brenner et al. 1997, Ho et al. 2001), Hodgkin's disease (Castenet et al. 1995), malignant melanoma (Modley et al. 1989), and meningioma (Richardson and Cohen 2000). The significance of these anecdotal observations remains to be determined and the authors do not routinely screen new SCLE patients for occult malignancy.

HLA-B8, DR3, DQwl/DQw2

Anti-Ro/SS-A

SCLE /

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SCLE/SSj Overlap Fig. 2. Immunogenetic associations of SCLE and Sjogren's syndrome

SCLE/SSj Overlap Fig. 2. Immunogenetic associations of SCLE and Sjogren's syndrome

SCLE has been associated with a myriad of other diseases. Polymorphic light eruption (PLE), an inherited photosensitivity disorder, has frequently been associated with SCLE and they may have a common genetic predisposition (Millard et al. 2001b). Two-thirds of SCLE cohorts develop PLE and PLE cohorts have an increased relative risk of SCLE (Millard et al. 2001b). Case reports of porphyria cutanea tarda (Camp and Davis 1997), Sweet's syndrome (Goette 1985), Crohn's disease (Ashworth 1992), gluten sensitive enteropathy (Messenger and Church 1986), and X-linked Chronic Granulomatous Disease Carrier Status (Cordoba-Guijarro et al. 2000) have been associated with SCLE. Because of the infrequency of the latter reports, they may be incidental.

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