Refractory Bullous Systemic Lupus Erythematosus Successfully Treated with Rituximab: A Case Report and Literature Review

Abstract

Bullous systemic lupus erythematosus (BSLE) is a rare blistering skin manifestation of systemic lupus erythematosus (SLE). Dapsone is reported to be helpful in mild-to-moderate BSLE cases; however, its use may be limited or prohibited due to particular complications such as drug hypersensitivity, dose-dependent hemolytic anemia, and other significant hematologic abnormalities. Rituximab, an anti-CD20 monoclonal antibody, has been reported with off-label use in BSLE patients, but data are still limited. Hence, our objective is to explore the efficacy of rituximab among these patients. Herein, we report a 21-year-old Thai woman presented with blistering eruption on the oral cavity, scalp, trunk, and extremities for 1 month. The investigations revealed a positive direct Coomb's test, an elevated erythrocyte sedimentation rate (ESR), and a positive antinuclear antibody (ANA). Skin biopsy showed focal interface dermatitis. Direct immunofluorescence (DIF) illustrated mixed linear and granular deposition of immunoglobulin (Ig)G, IgM, IgA, and C3 along the dermo-epidermal junction (DEJ). Enzyme-linked immunosorbent assay (ELISA) showed circulating antibodies to type VII collagen. She was diagnosed with severe BSLE and autoimmune hemolytic anemia (AIHA) refractory to several oral immunosuppressants but was successfully treated with rituximab. The authors also performed a review of the literature on prior BSLE cases managed with rituximab.

Keywords: anti-CD20 monoclonal antibody; autoimmune bullous diseases; bullous systemic lupus erythematosus; subepidermal blistering eruption; systemic lupus erythematosus.

Figure 1

A 21-year-old female diagnosed with severe BSLE and autoimmune hemolytic anemia. Multiple tense bullae on erythematous base at trunk and extremities.

Figure 2

Direct immunofluorescence, (a) mixed linear and granular deposition of IgG along dermo-epidermal junction (DEJ), (b) Granular deposition of IgM along DEJ and superficial blood vessel, (c and d) mixed linear and granular deposition the along DEJ and perifollicular granular deposition of C3.

Figure 3

Improvement of BSLE after rituximab therapy. One month after 2 intravenous infusions of rituximab 1000 mg with a 2-week interval apart, most of the lesions healed with post-inflammatory hyperpigmentation.