Bullous SLE: A Phenotypically Distinctive but Immunologically Heterogeneous Bullous Disorder.

Abstract

Bullous systemic lupus erythematosus (SLE) is a rare blistering disease with a distinctive combination of clinical, histologic and immunopathologic features that together constitute a unique bullous disease phenotype. There appear to be at least two immunologically distinct subtypes of bullous SLE characterized by the presence or absence of circulating and/or tissue-bound basement membrane zone autoantibodies that recognize type VII collagen. The two subtypes are not clearly distinguishable except by indirect immunofluorescence and/or direct immunoelectron microscopy. In patients without circulating antibodies, immunoelectron microscopy is required to distinguish between the two subtypes. Patients with autoantibodies to type VII collagen are similar but not identical to patients with epidermolysis bullosa acquisita--another bullous disease associated with autoantibodies to type VII collagen. Autoantibodies to type VII collagen in patients with bullous SLE is only one of several lines of evidence that indicate autoimmunity to that protein and susceptibility to SLE are associated phenomena. In addition, there is emerging evidence for an association between epidermolysis bullous acquisita and SLE. There is also evidence that autoantibodies to type VII collagen are pathogenic in bullous SLE (and epidermolysis bullosa acquisita) and that their production is regulated by the class II major histocompatibility complex DR beta 1 allele, 1501 and possibly other DR beta 1 alleles that share a similar sequence of amino acids in the second hyper-variable region.

Utility of direct immunofluorescence studies in subclassification of autoimmune sub-epidermal bullous diseases: a 2-year study in a tertiary care hospital

“…saltsplit DiF studies will distinguish between these entities. The patterns of immunoreactant deposition in bullous slE can be granular, linear and rarely fibrillar (23). The dermal floor pattern of indirect immunofluorescence on salt-split skin substrate is found in the sera of patients with both bullous slE and EBa (24).…”

Crusted lesions on the face of a child

“…Blister lesions can present as pemphigoid‐like eruptions with tense vesicles and bullae; alternatively, they are clustered like dermatitis herpetiformis; Nikolsky's sign is usually negative 3, 4. The diagnosis of BSLE requires the demonstration of autoantibodies against the BMZ protein, type VII collagen, or an immune electron microscopy examination revealing immunoglobulin deposition under the lamina densa and reticular lamina 5, 6…”

Bullous Systemic Lupus Erythematosus With Autoantibodies Recognizing Multiple Skin Basement Membrane Components, Bullous Pemphigoid Antigen 1, Laminin-5, Laminin-6, and Type VII Collagen

“…1999;135:569-573 B ULLOUS SYSTEMIC lupus erythematosus (BSLE) is an autoantibody-mediated blistering skin disease occurring in patients with systemic lupus erythematosus (SLE). [1][2][3][4][5][6][7][8][9] In addition to having clinical and laboratory findings that fulfill the American Rheumatism Association diagnostic critieria of SLE, 2 patients with BSLE have a generalized subepidermal blistering skin disease with immunoglobulin deposition along the skin basement membrane zone (BMZ).…”