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• W4383223893 abstract "A young woman with systemic lupus erythematosus on hydroxychloroquine, mycophenolate mofetil, and prednisone presented to the dermatology clinic with a 3-week history of a bullous eruption involving the face, oral mucosa, elbows, and hands (Figure 1). A skin biopsy with direct immunofluorescence revealed a neutrophil-rich subepidermal vesiculobullous dermatitis and linear deposition of immunoglobulin G (IgG), IgM, IgA, and fibrinogen at the basement membrane zone, consistent with bullous systemic lupus erythematosus (BSLE) (Supplemental Figure, available online at http://www.mayoclinicproceedings.org). Within 1 week, the bullae spread to the trunk and extremities, necessitating hospitalization (Figure 2). Laboratory testing revealed double-stranded DNA autoantibodies 5640 IU/mL, creatinine 0.6 mg/dL, and 2+ proteinuria. A subsequent renal biopsy was consistent with lupus nephritis. Dapsone was initiated and prednisone was changed to methylprednisolone for the treatment of lupus nephritis. The bullae abated by 2 weeks and re-epithelialized by 2 months.Figure 2Numerous tense and flaccid bullae on the back.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Bullous systemic lupus erythematosus is a rare disorder most often observed in young females of African descent.1Sprow G. Afarideh M. Dan J. Hedberg M.L. Werth V.P. Bullous systemic lupus erythematosus in females.Int J Womens Dermatol. 2022; 8e034Google Scholar It typically occurs in patients with pre-existing systemic lupus erythematosus, although it may present as the initial manifestation.2Fujimoto W. Hamada T. Yamada J. Matsuura H. Iwatsuki K. Bullous systemic lupus erythematosus as an initial manifestation of SLE.J Dermatol. 2005; 32: 1021-1027Crossref PubMed Scopus (47) Google Scholar Autoantibodies against type VII collagen in the basement membrane result in the subepidermal blisters found in BSLE.3Sebaratnam D.F. Murrell D.F. Bullous Systemic Lupus Erythematosus.Dermatol Clin. 2011; 29: 649-653Abstract Full Text Full Text PDF PubMed Scopus (31) Google Scholar Lesions typically begin as erythematous macules or inflammatory plaques that progress to bullae on sun-exposed and mucosal surfaces.3Sebaratnam D.F. Murrell D.F. Bullous Systemic Lupus Erythematosus.Dermatol Clin. 2011; 29: 649-653Abstract Full Text Full Text PDF PubMed Scopus (31) Google Scholar Up to 90% of BSLE cases have concomitant systemic involvement, most often with lupus nephritis, cytopenia, arthralgias, or neuropsychiatric lupus.4de Risi-Pugliese T. Cohen Aubart F. Haroche J. et al.Clinical, histological, immunological presentations and outcomes of bullous systemic lupus erythematosus: 10 New cases and a literature review of 118 cases.Semin Arthritis Rheum. 2018; 48: 83-89Crossref PubMed Scopus (41) Google Scholar Biopsy is essential for diagnosis of BSLE, with classic findings of papillary-tip neutrophil microabscesses and deposition of immunoglobulins, especially IgG, at the dermal-epidermal junction.5Vassileva S. Bullous systemic lupus erythematosus.Clin Dermatol. 2004; 22: 129-138Abstract Full Text Full Text PDF PubMed Scopus (90) Google Scholar Two punch biopsies should be performed, a lesional biopsy at the blister edge for hematoxylin and eosin staining and a perilesional biopsy for direct immunofluorescence. Dapsone is a highly effective treatment for BSLE; in severe or recalcitrant cases, steroids or immunosuppressants are viable alternatives.4de Risi-Pugliese T. Cohen Aubart F. Haroche J. et al.Clinical, histological, immunological presentations and outcomes of bullous systemic lupus erythematosus: 10 New cases and a literature review of 118 cases.Semin Arthritis Rheum. 2018; 48: 83-89Crossref PubMed Scopus (41) Google Scholar The authors report no potential competing interests. The authors thank Laura Ragmanauskaite, MD, for her care of the patient and photography." @default.
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• W4383223893 date "2023-07-01" @default.
• W4383223893 modified "2023-09-25" @default.
• W4383223893 title "Bullous Systemic Lupus Erythematosus With Lupus Nephritis" @default.
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• W4383223893 doi "https://doi.org/10.1016/j.mayocp.2023.03.002" @default.
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