Bullous Lesions

Bullous lesions occur infrequently in patients with SLE. Blister formation may develop in some lupus lesions, especially at the periphery. This is generally associated with the presence of anti-Ro/SSA antibodies. On rare occasions, blister formation, in all probability an extension of the microscopic separation at the dermal-epidermal junction, may become very extensive, especially in anti-Ro/SSA antibody-positive photosensitive patients with SLE, producing cutaneous manifestations similar to toxic epidermal necrolysis (Bielsa et al. 1987).

In addition, to this type of blister formation, an acquired form of epidermolysis bullosa acquisita has been described (Gammon et al. 1985, Gammon and Briggaman 1993). These patients with SLE are HLA-DR2 positive and possess in their sera autoantibodies reactive against type VII collagen. Clinically, these patients have widespread vesiculobullous eruptions. Immunofluorescence studies detect a thick, linear deposit of IgA, IgG, and C3 along the basement membrane zone. Using a 1 M NaCL split-skin technique, anti-basement membrane antibodies are found on the dermal side of the split. Immunoelectron microscopy studies demonstrate that the IgG deposits are below the lamina densa. These epidermolysis bullosa acquisita lesions may occur simultaneously with, or occur after the development of SLE.

A third pathophysiologic mechanism of blister formation in SLE has been described as a vesiculobullous eruption characterized histologically by the presence of neutrophilic microabscess formation in the dermal papillae reminiscent of dermatitis herpetiformis (Hall et al. 1982). Direct immunofluorescence of non-UV lightexposed normal-appearing skin revealed IgA, IgE, IgM, and C3 deposition along the dermal-epidermal junction. Immune complexes were demonstrated in the sera of several of these patients.

It seems likely that these blisters were the result of an immune complex-mediated vasculitis involving small blood vessels at the dermal-epidermal junction. It is theorized that the inflammatory infiltrate destroyed the integrity of the dermal-epidermal junction promoting blister formation. It should be noted that all patients had a dramatic response to diaminodiphenylsulfone (Dapsone).

In addition to these forms of bullous lesions, bullous pemphigoid, dermatitis her-petiformis, and pemphigus erythematosus have occasionally been reported in association with LE. The Senear-Usher syndrome (pemphigus erythematosus) is a rare condition characterized by patients having a combination of pemphigus foliaceus and SLE (Chorzelski et al. 1968, Cruz et al. 1987). Clinically, these patients most commonly demonstrate seborrheic dermatitis-like distribution of their pemphigus disease process. Histologically, an acantholytic process at the level of the stratum malpighii has been demonstrated, and these patients demonstrate pemphigus antibodies directed against desmoglein I. Serologic studies generally demonstrate the presence of antinuclear antibodies, but the presence of other antibodies, such as anti-dsDNA and anti-RNP, are unusual.

Based on the author's personal experience and a review of the literature, it seems that the coexistence of LE with other punitive autoimmune bullous diseases most likely represents the occurrence of two autoimmune diseases in patients who are genetically susceptible.

Finally, a few words should be stated regarding porphyria cutanea tarda, which has been reported in association with SLE, discoid LE, and SCLE (Callen and Ross 1981, Clemmenson and Thomasen 1982, Cram et al. 1973, Weatherhead and Adam 1985). It also should be noted that in addition to porphyria cutanea tarda, acute intermittent porphyria and variegate porphyria have also been found in association with LE.

Although studies published in the 1970 s suggested that porphyria cutanea tarda was a common association with SLE, it is now believed that a causative relationship between the two diseases is unlikely. It is suspected that the use of antimalarials in the treatment of lupus precipitates or aggravates the underlying porphyrin abnormality, producing manifestations of porphyria cutanea tarda.

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