Keshavamurthy, Chandana

ABSTRACT Cutaneous lupus erythematosus (CLE) is one of the most common manifestations of systemic lupus erythematosus (SLE), although it can manifest as an independent entity as well. Bullous systemic lupus erythematosus (BSLE) is a rare cutaneous manifestation of SLE presenting as tense vesiculobullous eruptions in a photosensitive distribution. Pathophysiology is secondary to autoantibodies against noncollagenous domain 1 and 2 (NC1 and NC2) type VII collagen, and histopathology reveals dense neutrophilic infiltration of the dermis with direct immunofluorescence showing IgG deposition at dermoepidermal junction. There is lack of data on available therapeutic options to treat BSLE, and varying responses to dapsone, methotrexate, azathioprine and corticosteroids have been reported. Belimumab, a fully humanised Change to Immunoglobulin G1λ (IgG1λ) monoclonal antibody targeting soluble B lymphocyte stimulator protein, was the first Food and Drug Administration-approved drug for SLE and has been reported to be effective for CLE. We present the case of a 41-year-old black female with SLE presenting with BSLE, who was successfully treated with corticosteroids and belimumab and did not experience disease relapse even after discontinuation of corticosteroids. To our knowledge, this is the first reported case of successful treatment of BSLE with belimumab, and further research can help determine the role of belimumab in the treatment of BSLE.