Bullous systemic lupus erythematosus (BSLE) is a rare autoimmune blistering disease in patients with a known diagnosis of systemic lupus erythematosus (SLE). It can be the presenting symptom of SLE in patients who do not carry a diagnosis. Patients with BSLE should be worked up for systemic involvement; according to one study of 118 patients, 50% of patients with BSLE have lupus nephritis, 45% have hematologic abnormalities, and 19% have neuropsychiatric involvement.

BSLE is caused by autoantibody formation against the noncollagenous type 1 and 2 (NC1 and NC2) components of Collagen VII (Col7), causing disruption and separation of the structure of the dermal-epidermal junction, with subsequent complement deposition, and recruitment of neutrophils. Col7 is the major component of the anchoring fibrils in the cutaneous basement membrane zone (BMZ).

BSLE is typically a disease of young adulthood, affecting those between 20 and 40 years of age, but it has also been reported in children and older adults. Similar to the sex predilection of SLE, BSLE affects more women than men.

BSLE typically presents as an abrupt onset of tense vesicles and blisters on an erythematous background or on normal skin. Urticarial plaques may be present. Face, trunk, and extensor surfaces of limbs are the most commonly involved body sites, but other body locations can be involved. BSLE may clinically mimic a range of autoimmune diseases, most commonly bullous pemphigoid (BP). Epidermolysis bullosa acquisita (EBA)-like, linear IgA bullous dermatosis (LABD)-like, and dermatitis herpetiformis (DH)-like presentations have also been noted.

Photodistribution or photoexacerbation has been reported. Intraoral blisters and erosions and blistering along the lip vermillion can be seen. Esophageal involvement with sloughing of the esophageal mucosa has rarely been reported.