Bullous systemic lupus erythematosus: revised criteria for diagnosis

Abstract

Summary Blistering in systemic lupus erythematosus has been divided into three groups.1 A specific subgroup of ‘bullous systemic lupus erythematosus’ has been defined by Gammon et al. on the basis of a number of criteria.2 From our experience of seven patients with bullous systemic lupus erythematosus, and after reviewing the literature, we suggest that the current classification is too narrow. Our patients displayed clinical and immunohistolegical (based on direct and indirect immunofluorescence and Western immunoblotting) heterogeneity. Sera from two patients bound to epidermal epitopes in sodium chloride‐split skin, but immunoblotting was negative. In neither of these patients could the target antigen be type VII collagen, the only antigen identified as pathogenic in this disease. Patients with epidermal binding should not be excluded from a diagnosis of bullous systemic lupus erythematosus. SLE is a disease in which there is a genetic predisposition to form antibodies to type VTI collagen, along with other autoantibodies, many of which may be implicated in blistering. We suggest that the criteria for the diagnosis of BSLE should be revised. We define this disease as an acquired subepidermal blistering disease in a patient with SLE, in which immune reactants are present at the basement membrane zone on either direct or indirect immunofluorescence.

A patient with multiple blisters in the skin and mucous membranes

“…Current immunopathologic criteria of bullous SLE are similar to those of EBA and include: immunoglobulin and complement deposition at the basement membrane zone (BMZ) on direct immunofluorescence (DIF), and immune deposits ultrastructurally localised on or beneath the lamina densa 3. Although both EBA and bullous SLE are autoimmune diseases, and often have a common human leucocyte antigen (HLA) major histocompatibility (MHC) class II cell surface protein, HLA-DR2, the extent of the relationship between them is not known, although patients with both diseases have been reported 4.…”

Complement and cutaneous autoimmune blistering diseases

“…Experiment models demonstrate that C5a is involved in tissue damage, and there has been some success in using anti-C5a antibodies to treat the disease [1]. In some cases BSLE is associated with autoantibodies directed against type VII collagen [60] and C3 is detected in the BMZ [61,62]. IgG autoantibodies from BSLE induce leukocyte-dependent dermal-epidermal junction separation in an ex vivo human skin cryosections model [63].…”

Systemischer Lupus erythematodes

“…Die Blasen heilen mit Hyperpigmentierung, oft unter Ausbildung von Milien und meist mit zarten Narben [21][22][23][24][25].…”