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Bullous systemic lupus erythematosus in females
Bullous systemic lupus erythematosus (BSLE) is a rare blistering presentation of systemic lupus erythematosus, typically affecting women with the highest incidence in those of African descent. The key pathogenic insult includes the formation of autoantibodies against type VII collagen, which weaken...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Lippincott Williams & Wilkins
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9324630/ https://www.ncbi.nlm.nih.gov/pubmed/35923586 http://dx.doi.org/10.1097/JW9.0000000000000034 |
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author | Sprow, Grant Afarideh, Mohsen Dan, Joshua Hedberg, Matthew L. Werth, Victoria P. |
author_facet | Sprow, Grant Afarideh, Mohsen Dan, Joshua Hedberg, Matthew L. Werth, Victoria P. |
author_sort | Sprow, Grant |
collection | PubMed |
description | Bullous systemic lupus erythematosus (BSLE) is a rare blistering presentation of systemic lupus erythematosus, typically affecting women with the highest incidence in those of African descent. The key pathogenic insult includes the formation of autoantibodies against type VII collagen, which weaken the basement membrane zone and lead to the formation of subepidermal blisters. The acute vesiculobullous eruptions in BSLE generally tend to affect photo-distributed areas, although they can arise unrelated to sun exposure (eg, mucous membranes, axillae). The bullae can arise from erythematous macules, inflammatory plaques, or previously normal skin. Their appearance can range from small, grouped vesicles reminiscent of lesions in dermatitis herpetiformis to large, tense blisters, similar to bullous pemphigoid. Internal organ involvement occurs in up to 90% of those affected. This mostly includes lupus nephritis (classes III–V, lifetime prevalence of up to 90%), arthralgias/arthritis, and cytopenias, while serositis and neuropsychiatric involvement are rare. First-line management with dapsone should be considered in mild disease with stable underlying systemic lupus erythematosus. As discussed in this review, the off-label use of rituximab (an anti-CD20 B-cell depleting agent) has been shown to be safe and effective in several refractory cases of BSLE unresponsive to dapsone, glucocorticoids, or steroid-sparing immunosuppressants. |
format | Online Article Text |
id | pubmed-9324630 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Lippincott Williams & Wilkins |
record_format | MEDLINE/PubMed |
spelling | pubmed-93246302022-08-02 Bullous systemic lupus erythematosus in females Sprow, Grant Afarideh, Mohsen Dan, Joshua Hedberg, Matthew L. Werth, Victoria P. Int J Womens Dermatol Review Bullous systemic lupus erythematosus (BSLE) is a rare blistering presentation of systemic lupus erythematosus, typically affecting women with the highest incidence in those of African descent. The key pathogenic insult includes the formation of autoantibodies against type VII collagen, which weaken the basement membrane zone and lead to the formation of subepidermal blisters. The acute vesiculobullous eruptions in BSLE generally tend to affect photo-distributed areas, although they can arise unrelated to sun exposure (eg, mucous membranes, axillae). The bullae can arise from erythematous macules, inflammatory plaques, or previously normal skin. Their appearance can range from small, grouped vesicles reminiscent of lesions in dermatitis herpetiformis to large, tense blisters, similar to bullous pemphigoid. Internal organ involvement occurs in up to 90% of those affected. This mostly includes lupus nephritis (classes III–V, lifetime prevalence of up to 90%), arthralgias/arthritis, and cytopenias, while serositis and neuropsychiatric involvement are rare. First-line management with dapsone should be considered in mild disease with stable underlying systemic lupus erythematosus. As discussed in this review, the off-label use of rituximab (an anti-CD20 B-cell depleting agent) has been shown to be safe and effective in several refractory cases of BSLE unresponsive to dapsone, glucocorticoids, or steroid-sparing immunosuppressants. Lippincott Williams & Wilkins 2022-07-27 /pmc/articles/PMC9324630/ /pubmed/35923586 http://dx.doi.org/10.1097/JW9.0000000000000034 Text en Written work prepared by employees of the Federal Government as part of their official duties is, under the U.S. Copyright Act, a “work of the United States Government” for which copyright protection under Title 17 of the United States Code is not Written work prepared by employees of the Federal Government as part of their official duties is, under the U.S. Copyright Act, a “work of the United States Government” for which copyright protection under Title 17 of the United States Code is not available. As such, copyright does not extend to the contributions of employees of the Federal Government. |
spellingShingle | Review Sprow, Grant Afarideh, Mohsen Dan, Joshua Hedberg, Matthew L. Werth, Victoria P. Bullous systemic lupus erythematosus in females |
title | Bullous systemic lupus erythematosus in females |
title_full | Bullous systemic lupus erythematosus in females |
title_fullStr | Bullous systemic lupus erythematosus in females |
title_full_unstemmed | Bullous systemic lupus erythematosus in females |
title_short | Bullous systemic lupus erythematosus in females |
title_sort | bullous systemic lupus erythematosus in females |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9324630/ https://www.ncbi.nlm.nih.gov/pubmed/35923586 http://dx.doi.org/10.1097/JW9.0000000000000034 |
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