SCLE, a subset with specific clinical and serologic features occurring preferentially in white females, was first discussed as a distinct entity by Gilliam in 1977 (Gilliam 1977), with expanded descriptions in 1979 (Sontheimer et al. 1979), 1981 (Gilliam and Sontheimer 1981), and 1982 (Gilliam and Sontheimer 1982). This subtype shows a significant coincidence with HLA-DR2 or -DR3, and patients with overlapping manifestations of Sjogren's syndrome or high levels of anti-Ro/SSA antibodies are more likely to have HLA-B8, -DR3, -DRw6, -DRw52, and -DQ2 (Sontheimer et al. 1981). Certain drugs, especially hydrochlorothiazide, angiotensin-converting enzyme inhibitors, and calcium channel blockers have been associated with the onset and/or exacerbation of this disease (Reed et al. 1985, Srivastava et al. 2003). In recent years, terbinafine has also been reported to induce SCLE with high titers of antinuclear antibodies (ANAs) and antihistone antibodies in genetically susceptible persons (Bonsmann et al. 2001, Callen et al. 2001). Furthermore, most patients with this subtype are sensitive to sunlight and exposure to UV irradiation can precipitate or aggravate SCLE lesions (Kuhn et al. 2001a). Patients with SCLE typically have prominent cutaneous and musculoskeletal complaints but generally do not develop a severe
Fig. 6.4. Papulosquamous subacute cutaneous lupus erythematosus (SCLE). Psoriasiform lesions with superficial scale and the tendency for individual lesions to merge into a vetiform pattern
systemic disease, and only half of them have four or more of the American Rheumatism Association (ARA) criteria for the diagnosis of systemic lupus erythematosus (SLE) (Cohen and Crosby 1994, Crowson and Magro 2001, Tan et al. 1982). Therefore, SCLE can be considered a relatively benign illness that is intermediate in severity between ACLE and CCLE (Sontheimer 1989).
Initially, SCLE lesions present with erythematous macules and papules that evolve into scaly papulosquamous or annular/polycyclic plaques (Sontheimer et al. 1981). Approximately 50% of patients have predominantly papulosquamous or psoriasiform lesions (Fig. 6.4), and the other half have the annular/polycyclic type (Fig. 6.5); a few patients may develop both forms of lesions (Sontheimer 1985a, Sontheimer et al. 1979). However, some groups have observed a predominance of the papulosquamous lesions, whereas others have noted an abundance of the annular/polycyclic type (Callen and Klein 1988, Chlebus et al. 1998, Cohen and Crosby 1994, David-Bajar 1993, Fabbri et al. 1990, Herrero et al. 1988, Molad et al. 1987). One recent study found that 42% of the patients with SCLE studied exhibit the annular/polycyclic form, 39% had the papulosquamous form, and 16% showed both manifestations (Parodi et al. 2000). Generally, lesions of this subtype heal without scarring but can leave long-lasting and permanent vitiligo-like pigmentary changes as a "clue" for the clinical diagnosis (Fig. 6.6) (Milde and Goerz 1994). SCLE has a characteristic distribution of lesions in sun-exposed areas, in particular the upper chest and back, the deltoid aspect of the shoulders, the extensor surface of the arms, and less commonly the face or scalp. In the study by Parodi et al. (Parodi et al. 2000), the neck was affected in 83% of patients, with 66% exhibiting lesions on the face, 39% on the extensor arms, 21% on the dorsal hands, 16% on the lower limbs, and 12% on the scalp. Interestingly, 27%-100% of patients with SCLE have been reported to be abnormally photosensitive, and experimental studies showed positive phototest results in 63% after UVA or
UVB irradiation (Kuhn et al. 2001a). However, some ethnic groups, such as Japanese and Chinese, seem to be less photosensitive (Nishikawa and Provost 1991, Shou-yi et al. 1987). A prevalence of polymorphous light eruption of 60%-70% has also been demonstrated in patients with SCLE, as well as phototoxic reactions when photosensitizing medications were prescribed (Millard et al. 2000).
Several other skin lesions that are not specific for LE have been described in patients with SCLE (Parodi et al. 2000, Sontheimer 1989). The most frequently encountered of these include nonscarring alopecia, painless mucous membrane lesions, livedo reticularis, periungual telangiectasias, and Raynaud's phenomenon (Callen et al. 1986, Callen and Klein 1988, David et al. 1984, Herrero et al. 1988, Molad et al. 1987, Sanchez-Perez et al. 1993, Sontheimer 1985a). Cutaneous vasculitis of the lower extremities is a frequent finding in anti-Ro/SSA antibody-positive patients with SCLE also described under the rubric of Sjogren's syndrome/LE overlap syndrome (Provost et al. 1988). Furthermore, cutaneous calcinosis maybe seen rarely in patients with SCLE, and HPV-11-associated squamous cell carcinomas of the skin were noted in one patient with SCLE (Cohen et al. 1992). In one additional case, annular/poly-cyclic SCLE lesions were reported over time to progress to plaques of morphea (Rao et al. 1990).
Patients with SCLE may also develop localized facial ACLE, which has been seen in 7%-100% of patients (David et al. 1984, Fabbri et al. 2003, Molad et al. 1987, Shou-yi et al. 1987, Sontheimer 1985a). However, ACLE skin lesions tend to be more transient, to heal less often with pigmentary changes, and to be more edematous and less scaly than SCLE lesions. ACLE more commonly affects the malar areas of the face; in general, SCLE involves the face much less often. Several reports have also noted that up to 29% of patients with SCLE manifest DLE lesions during their clinical course and, interestingly, 19% of the original cohort of patients with SCLE had classical DLE
Fig. 6.6. Hypopigmentation in subacute cutaneous lupus erythematosus (SCLE). Permanent vitiligo-like depigmentation in the face of a patient with SCLE
(Callen and Klein 1988, David et al. 1984, Molad et al. 1987, Shou-yi et al. 1987, Sontheimer 1985a, Sontheimer et al. 1979). These lesions can predate the onset of SCLE lesions; however, DLE lesions are generally associated with a greater degree of pigmentary changes, may display atrophic dermal scarring, and are more characteristically associated with follicular plugging and adherent scaling than SCLE lesions. Furthermore, DLE lesions are characteristically indurated, whereas SCLE lesions are not (David-Bajar et al. 1992).
In addition to the common forms of SCLE, several unusual varieties of this subtype have been reported. Infrequently, SCLE lesions present initially with an appearance of erythema multiforme, which can simulate Rowell's syndrome (erythema mul-tiforme-like lesions occurring in patients with SLE in the presence of anti-La/SSB autoantibodies) (Rowell et al. 1963, Sontheimer 1985b). Lyon et al. (Lyon et al. 1998) reported two cases of delayed diagnosis of SCLE because of the clinical and histologic similarities between SCLE and erythema multiforme. Furthermore, lesions similar to erythema annulare centrifugum can be seen in some patients, and, as a result of hyperacute basal cell layer injury, on rare occasions, the active edge of annular SCLE lesions undergoes a vesicular change that breaks down to produce a striking crusted appearance (Grant 1981, Wechsler and Stavrides 1982). On at least one occasion such lesions have progressed to mimic toxic epidermal necrolysis (Bielsa et al. 1987), and in a study by Herrero et al. (Herrero et al. 1988), vesiculobullous changes were present in 38% of the SCLE population, which coincided histologically with focal areas of necrosis. In 1988, one patient with SCLE was reported to initially present exfoliative erythroderma (DeSpain and Clark 1988) and this was also noted more recently by Mutasim (Mutasim 2003). Other patients presented with a curious acral distribution of annular lesions (Scheinman 1994) or a form of SCLE with widespread plaques (Tsutsui et al. 1996). Pityriasisform (Caproni et al. 2001,Hymes et al. 1986) and exan-
thematous (Sontheimer 1985b) variants of SCLE have been mentioned anecdotally on rare occasions. In addition, follicular erythematous lesions are occasionally seen in patients with SCLE, and it has been reported to be associated with generalized poikiloderma (Pramatarov et al. 2000).
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