Epidermolysis bullosa acquisita occurring in association with systemic lupus erythematosus.

A 77-year-old retired male physician with a 6-year history of systemic lupus erythematosus (SLE) developed a mechanobullous eruption, the features of which were clinically and immunopathologically consistent with a diagnosis of 'classical' epidermolysis bullosa acquisita (EBA). As EBA shares immunopathological findings with a number of cases reported as the 'bullous eruption of SLE', the clinical findings commonly recognized as 'classical EBA' may, in patients with SLE, represent a specific subset of the bullous eruption of SLE rather than a separate diagnostic entity. There are few reports in the literature describing classical EBA in patients with SLE. Findings in this patient add further support to the suggestion that EBA occurring in association with SLE, represents a subset of the bullous eruption of SLE, the clinical features of which may be modified by genetic susceptibility or disease activity.