Bullous Lesions in Lupus Erythematosus

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The frequency of bullous lesions associated with LE (BLE) is low, and it has been reported that less than 5% of patients with SLE and skin changes had chronic vesicu-lobullous lesions (Gammon and Briggaman 1993, Yell and Wojnarowska 1997). Confusion has always existed concerning the nosology and classification of the bullous skin changes that can occur in patients with LE, and there has been no consensus in this area for a long time. In 1997, Sontheimer (Sontheimer 1997) divided the various types of vesicular and bullous skin lesions that can be encountered in patients with LE into those that have or do not have LE-specific pathology and proposed the following classification scheme:

(1) Bullae may develop in LE-specific skin lesions such as ACLE or other cutaneous forms of LE as a direct extension of the vacuolar degeneration of the epidermal basal layer. Skin cleavage occurs as a result of dissolution of the basal cell layer, resulting in subepidermal cleavage that may have the clinical appearance of toxic epidermal necrolysis. Anti-Ro/SSA antibody-positive patients seem to be at increased risk of developing this complication following UV light exposure (Gilliam et al. 1985, Kulick et al. 1983). Documentation of LE as the causal factor in this type of bullous skin change can be difficult because such patients are also frequently taking systemic medication and toxic epidermal necrolysis, therefore, also commonly develops as a result of drug hypersensitivity reaction. In patients with SCLE, vesiculobullous changes may also occur at the active advancing edge of annular lesions (Grant 1981, Sontheimer 1985b, Wechsler and Stavrides 1982), and subepidermal bullous changes have also been reported to be present in DLE lesions; however, these changes seem to be extremely rare (Nagy and Balogh 1961).

(2) On unusual occasions,bullous eruptions or vesiculobullous lesions, unassociated with LE skin lesions, occur in patients with SLE (Bacman et al. 2004, Camisa and Sharma 1983, Gammon et al. 1985, Hall et al. 1982, Olansky et al. 1982, Penneys and Wiley 1979). These lesions predominantly involve flexural or extensor skin (Fig. 6.19), the upper trunk, and the supraclavicular regions, but the face and the mucosal membranes are also predilection sites. The bullae arise on erythematous or normal skin, tend to be tense, and may approach the size of blisters in

Fig. 6.19. Bullous skin lesions associated with lupus erythematosus (BLE). Firm bullous lesions on the extensor aspect of the lower arm in a patient with SLE

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bullous pemphigoid or may resemble the vesiculobullous lesions of dermatitis herpetiformis. If the bullous lesions rupture, they leave erosions, crusts, and pigmentary changes, and when they regress, scars, milium cysts, or calcinosis cutis can remain (Eckman and Mutasim 2002). In this form of bullous skin lesions, the activity of blistering may or may not coincide with the activity of the patient's systemic disease, and occasionally, mild and serious cases associated with organ-threatening disease have been reported (Gammon and Briggaman 1993, Sontheimer 1997). An important and interesting feature of these patients is also the dramatic response to treatment with dapsone even with very small doses (Hall et al. 1982, Yung and Oakley 2000). However, the drug could not be tapered and discontinued without rapid recurrence of the lesions. In a recent study, the efficacy of methotrexate in bullous SLE has also been reported in one patient (Malcangi et al. 2003). Histologically, the subepidermal blisters demonstrate neutrophilic microabscesses in dermal papillae along with a perivascular and periadnexal infiltration consisting of lymphocytes and neutrophils (Megahed 2004). Direct immunofluorescence shows linear or granular deposits of IgG, IgA, and/or IgM and complement along the basement membrane zone, and immunoelectron microscopic studies demonstrate IgG deposits at or below the lamina densa (Gammon and Briggaman 1993, Yell and Wojnarowska 1997). Furthermore, indirect immunofluorescent studies are positive using a 1 M NaCL split skin demonstrating mostly dermal binding (Gammon et al. 1985), and Western blot analysis showed that these autoantibodies bind to the noncollagenous portion of type VII collagen (Shirahama et al. 1998). These data further demonstrated that the autoantibodies in this bullous form of LE bind to the same epitopes as described in epidermolysis bullosa (Barton et al. 1986, Lapiere et al. 1993). Prior to these studies, two other groups had performed acid elution studies on skin of patients with SLE, demonstrating autoantibodies reactive against human skin (Landry and Sams 1974, Nicholas and Gilliam 1981, 1982). It is highly likely that these investigators were dealing with autoantibodies reactive against type VII collagen. In more recent studies, Chan et al. (Chan et al. 1999) concluded that patients with bullous SLE may have autoantibodies to multiple basement membrane components, such as bullous pemphigoid antigen-1, laminin-5, and laminin-6. Further-more,Yell and Wojnarowska (Yell and Wojnarowska 1997) demonstrated that two of seven patients also showed epidermal binding and, in one case, this was also associated with dermal binding.

In addition, a variety of primarily blistering diseases have been anecdotically reported to occur in patients with cutaneous and systemic manifestations of LE, in particular, dermatitis herpetiformis (Davies et al. 1976), bullous pemphigoid (Miller et al. 1978), pemphigus erythematosus (Chorzelski et al. 1968), pemphigus foliaceus (Blanchet et al. 1981), epidermolysis bullosa acquisita (Dotson et al. 1981), and linear IgA dermatoses (Lau et al. 1991). A non-autoimmune blistering skin disease, por-phyria cutaneous tarda, has also been reported in patients with different forms of LE (Gibson and McEvoy 1998, Weatherhead and Adam 1985); however, this association could also be merely fortuitous.

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