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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...

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Detalles Bibliográficos
Autores principales: Ye, X, Warrier, S, Nejad, K J, Parasyn, A D
Formato: Online Artículo Texto
Lenguaje:English
Publicado: JSCR Publishing Ltd 2012
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
Descripción
Sumario: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.