Diseases title

Bullous systemic lupus erythematosus )

Orpha Number: 46489

Bullous systemic lupus erythematosus (BSLE) is a generalized subepidermal blistering disease occurring in patients with systemic lupus erythematosus. It is a rare disease with approximately 70 cases reported in the literature so far and an estimated annual incidence of 1 per 5 millions. BSLE usually occurs in young adults but onset in older patients has been reported in a few isolated cases. The vesiculobullous eruption most often occurs on erythematous skin but may also occur on normal skin and is most commonly found on the upper trunk, face and neck. BSLE is an autoimmune disease associated with the production of autoantibodies against basement membrane zone antigens. Three immunologically distinct forms of BSLE can be distinguished depending on the type and localisation of these autoantibodies, with type I BSLE (characterized by the presence of antibodies to type VII collagen) being the most frequent. DSLE can generally be treated effectively with dapsone and resolves in less than 1 year.
source: Orphanet