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'.)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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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