BULLOUS SYSTEMIC LUPUS-ERYTHEMATOSUS - REVISED CRITERIA FOR DIAGNOSIS

Blistering in systemic lupus erythematosus has been divided into three groups.(1) A specific subgroup of bullous systemic lupus erythematosu s' has been defined by Gammon et al. on the basis of a number of crite ria.(2) From our experience of seven patients with bullous systemic lu pus erythematosus, and after reviewing the literature, we suggest that the current classification is too narrow, Our patients displayed clin ical and immunohistological (based on direct and indirect immunofluore scence and Western immunoblotting) heterogeneity, Sera from two patien ts bound to epidermal epitopes in sodium chloride-split skin, but immu noblotting was negative, In neither of these patients could the target antigen be type VII collagen, the only antigen identified as pathogen ic in this disease. Patients with epidermal binding should not be excl uded from a diagnosis of bullous systemic lupus erythematosus. SLE is a disease in which there is a genetic predisposition to form antibodie s to type VII 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 acq uired subepidermal blistering disease in a patient with SLE, in which immune reactants are present at the basement membrane zone on either d irect or indirect immunofluorescence.