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Subcutaneous spreading squamous cell carcinoma in a patient with epidermolysis bullosa
Epidermolysis Bullosa (EB) is a complex group of genetic disorders characterised by mechanical fragility in the basement membrane zone. Affected individuals experience significant morbidity and mortality, most commonly from cutaneous malignancies. In fact, 90.1% of EB patients develop Squamous Cell...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
JSCR Publishing Ltd
2012
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3649581/ https://www.ncbi.nlm.nih.gov/pubmed/24960768 http://dx.doi.org/10.1093/jscr/2012.8.10 |
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author | Ye, X Warrier, S Nejad, K J Parasyn, A D |
author_facet | Ye, X Warrier, S Nejad, K J Parasyn, A D |
author_sort | Ye, X |
collection | PubMed |
description | Epidermolysis Bullosa (EB) is a complex group of genetic disorders characterised by mechanical fragility in the basement membrane zone. Affected individuals experience significant morbidity and mortality, most commonly from cutaneous malignancies. In fact, 90.1% of EB patients develop Squamous Cell Carcinoma (SCC) before the age of 55, 80% of whom die within 5 years of diagnosis. Furthermore, the management of cutaneous malignancies in EB is fraught with challenges given the atypical presentations of malignancies and the co-existence of systemic co-morbidities. To illustrate the common pearls and pitfalls of managing EB in the perioperative setting, we present a case of SCC of the left forefoot which spread through a natural plane of weakness in the dermal-epidermal junction as a complication of the congenital weakness in the area resulting from EB. |
format | Online Article Text |
id | pubmed-3649581 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2012 |
publisher | JSCR Publishing Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-36495812013-05-14 Subcutaneous spreading squamous cell carcinoma in a patient with epidermolysis bullosa Ye, X Warrier, S Nejad, K J Parasyn, A D J Surg Case Rep Plastic Surgery Epidermolysis Bullosa (EB) is a complex group of genetic disorders characterised by mechanical fragility in the basement membrane zone. Affected individuals experience significant morbidity and mortality, most commonly from cutaneous malignancies. In fact, 90.1% of EB patients develop Squamous Cell Carcinoma (SCC) before the age of 55, 80% of whom die within 5 years of diagnosis. Furthermore, the management of cutaneous malignancies in EB is fraught with challenges given the atypical presentations of malignancies and the co-existence of systemic co-morbidities. To illustrate the common pearls and pitfalls of managing EB in the perioperative setting, we present a case of SCC of the left forefoot which spread through a natural plane of weakness in the dermal-epidermal junction as a complication of the congenital weakness in the area resulting from EB. JSCR Publishing Ltd 2012-08-01 /pmc/articles/PMC3649581/ /pubmed/24960768 http://dx.doi.org/10.1093/jscr/2012.8.10 Text en © JSCR |
spellingShingle | Plastic Surgery Ye, X Warrier, S Nejad, K J Parasyn, A D Subcutaneous spreading squamous cell carcinoma in a patient with epidermolysis bullosa |
title | Subcutaneous spreading squamous cell carcinoma in a patient with epidermolysis bullosa |
title_full | Subcutaneous spreading squamous cell carcinoma in a patient with epidermolysis bullosa |
title_fullStr | Subcutaneous spreading squamous cell carcinoma in a patient with epidermolysis bullosa |
title_full_unstemmed | Subcutaneous spreading squamous cell carcinoma in a patient with epidermolysis bullosa |
title_short | Subcutaneous spreading squamous cell carcinoma in a patient with epidermolysis bullosa |
title_sort | subcutaneous spreading squamous cell carcinoma in a patient with epidermolysis bullosa |
topic | Plastic Surgery |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3649581/ https://www.ncbi.nlm.nih.gov/pubmed/24960768 http://dx.doi.org/10.1093/jscr/2012.8.10 |
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