Bullous systemic lupus erythematosus
- Dan Lipsker, MD, PhD
Dan Lipsker, MD, PhD
- Medical Faculty
- University of Strasbourg
Bullous systemic lupus erythematosus (BSLE) is a rare autoimmune blistering disorder that typically manifests as an acute vesiculobullous eruption in a patient with known systemic lupus erythematosus (SLE). Rarely, BSLE is the initial clinical manifestation of SLE.
The differential diagnosis of blistering in patients with SLE is broad; therefore, careful assessment is necessary to confirm the diagnosis. The classic histologic and immunofluorescence findings of BSLE are separation within the dermal-epidermal junction, a neutrophilic infiltrate in the superficial dermis, immunoglobulin G (IgG) deposition at the dermal-epidermal junction, and antibody deposition on the dermal side of basement membrane zone-split skin. Anti-collagen VII autoantibodies are the serologic marker of BSLE. A rapid response to dapsone therapy is characteristic.
The clinical manifestations, diagnosis, and management of BSLE will be reviewed here. An overview of blistering skin disorders and a summary of the cutaneous manifestations of lupus erythematosus are provided separately. (See "Approach to the patient with cutaneous blisters" and "Overview of cutaneous lupus erythematosus".)
BSLE is a rare disorder for which epidemiologic data are limited [1,2]. In accordance with the epidemiology of systemic lupus erythematosus, BSLE is more frequent in females than in males. BSLE usually occurs in adults between the ages of 20 and 40 years; however, children and older adults also may be affected .
The clinical manifestations of BSLE result from the disruption of epidermal-dermal adhesion secondary to antibody formation against type VII collagen, the major component of the anchoring fibrils in the cutaneous basement membrane zone (figure 1). Anchoring fibrils play a critical role in the attachment of the epidermis to the dermis. (See "Epidermolysis bullosa acquisita", section on 'Type VII collagen'.)
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- ASSOCIATION WITH SYSTEMIC LUPUS ERYTHEMATOSUS
- CLINICAL MANIFESTATIONS
- Diagnostic criteria
- Our approach
- Diagnostic tests
- - Histopathology
- - Direct immunofluorescence
- - Indirect immunofluorescence
- - Antigen-specific serologic testing
- - Immunoelectron microscopy
- DIFFERENTIAL DIAGNOSIS
- Autoimmune blistering diseases
- Other disorders
- First-line treatment
- Refractory disease
- SUMMARY AND RECOMMENDATIONS