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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...

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Autores principales: Sprow, Grant, Afarideh, Mohsen, Dan, Joshua, Hedberg, Matthew L., Werth, Victoria P.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2022
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.
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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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